Professor Albert Ong

DM, MA, FRCP

Department of Infection, Immunity and Cardiovascular Disease

Professor of Renal Medicine

a.ong@sheffield.ac.uk
+44 114 215 9542

+44 114 271 1863 (fax)

The Medical School

Full contact details

Professor Albert Ong
Department of Infection, Immunity and Cardiovascular Disease
The Medical School
Beech Hill Road
Sheffield
S10 2RX

Profile

For enquiries, please contact – iicd-operational@sheffield.ac.uk

Albert Ong is Professor of Renal Medicine, Head of Academic Nephrology and Deputy Director for Clinical Academic Training at the University of Sheffield. He was educated at the University of Oxford and completed postgraduate medical training at University College London and Oxford.

Following doctoral studies in cell biology at UCL, he completed a postdoctoral fellowship in genetics at Oxford (KRUK Senior Research Fellow, Harris lab, Weatherall Institute of Molecular Medicine) before taking up a new Faculty position at the University of Sheffield. He was later awarded a major Wellcome Trust Research Leave Award to establish an independent research programme at Sheffield where his main focus has been to investigate the molecular basis of cyst formation, drug discovery and translational medicine in ADPKD.

He served as the main clinical expert to the NICE appraisal panel for the use of Tolvaptan in ADPKD and chaired the Renal Association Working Group which developed the first clinical guidance for its adoption and use in the UK. He is Chief Investigator of the PKD International Consortium (CYSTic) and serves on several international editorial boards, steering groups and scientific advisory committees including the ERA-EDTA Scientific Advisory Board, the ISN Fellowship Committee and the Mayo Clinic Translational PKD Centre (USA).

Research interests

The major research goals of my laboratory have been to define the molecular basis of autosomal dominant polycystic kidney disease (ADPKD), discover, develop and test new treatments and to improve the patient care pathway. We have defined the critical molecular interactions necessary for the formation, trafficking, regulation and function of the polycystin-1/polycystin-2 ADPKD protein complex and have identified several key binding partners, changes in cellular function and signalling resulting from the disease phenotype.

These findings are being used to develop new therapeutic approaches in experimental models to inhibit cyst formation or expansion with the ultimate goal of clinical translation. The clinical research programme has focussed on improving the patient pathway for patients and families with ADPKD. I am a founding member of the European ADPKD Forum which seeks to improve the multidisciplinary care for ADPKD patients across Europe through the adoption of agreed standards and an accepted patient care pathway.

Among several initiatives, we established the first UKGTN-approved comprehensive genetic diagnostic service for PKD1 and PKD2 in collaboration with the Sheffield Genetic Diagnostic Service in 2009 which has developed into an internationally used NGS panel for cystic diseases. We are an integral part of the Yorkshire and Humber Genome Medicine Centre (GMC) and the UK Renal GeCIP investigating genetic causes of rare kidney diseases as part of the UK 100K Genomes Project. We have also developed a new genetic psychosocial risk instrument for PKD (GPRI-PKD) and a semi-automated method for measuring total kidney volume from MRI (Sheffield TKV Tool).

Current projects:

Understanding the molecular basis of polycystic kidney disease.
Identifying new therapeutic targets for ADPKD in experimental models.
Improving diagnosis, prognosis and clinical outcomes in ADPKD.

Publications

Show: Featured publications All publications

Featured publications

Journal articles

Ong ACM & Gansevoort RT (2021) TAMEing ADPKD with metformin : safe and effective?. Kidney International, 100(3), 513-515.
Durkie M, Chong J, Valluru MK, Harris PC & Ong ACM (2020) Biallelic inheritance of hypomorphic PKD1 variants is highly prevalent in very early onset polycystic kidney disease. Genetics in Medicine.
Streets AJ, Prosseda PP & Ong ACM (2020) Polycystin-1 regulates ARHGAP35-dependent centrosomal RhoA activation and ROCK signaling. JCI Insight, 5(16).
Torres VE & Ong ACM (2020) Cellular signaling in PKD: foreword. Cellular Signalling, 71. View this article in WRRO
Lannoy M, Valluru MK, Chang L, Abdela-Ali F, Peters DJM, Streets AJ & Ong ACM (2020) The positive effect of selective prostaglandin E2 receptor EP2 and EP4 blockade on cystogenesis in vitro is counteracted by increased kidney inflammation in vivo. Kidney International. View this article in WRRO
Magayr TA, Song X, Streets AJ, Vergoz L, Chang L, Valluru MK, Yap HL, Lannoy M, Haghighi A, Simms RJ , Tam FWK et al (2020) Global microRNA profiling in human urinary exosomes reveals novel disease biomarkers and cellular pathways for Autosomal Dominant Polycystic Kidney Disease. Kidney International. View this article in WRRO
Ong ACM (2018) Tolvaptan slows disease progression in late-stage ADPKD. Nature Reviews Nephrology, 14(3), 146-148.
Feng S, Streets AJ, Nesin V, Tran U, Nie H, Onopiuk M, Wessely O, Tsiokas L & Ong A (2017) The Sorting Nexin 3 Retromer Pathway Regulates the Cell Surface Localization and Activity of a Wnt-Activated Polycystin Channel Complex. Journal of the American Society of Nephrology : JASN, 28(10), 2973-2984. View this article in WRRO
Ong ACM (2017) Making sense of polycystic kidney disease. The Lancet, 389(10081), 1780-1782.
Xu Y, Streets AJ, Hounslow AM, Tran U, Jean-Alphonse F, Needham AJ, Vilardaga J-P, Wessely O, Williamson MP & Ong ACM (2016) The Polycystin-1, Lipoxygenase, and α-Toxin Domain Regulates Polycystin-1 Trafficking. Journal of the American Society of Nephrology, 27(4), 1159-1173.
Ong ACM & Harris PC (2015) A polycystin-centric view of cyst formation and disease: the polycystins revisited. Kidney International, 88(4), 699-710. View this article in WRRO
Ong ACM, Devuyst O, Knebelmann B & Walz G (2015) Autosomal dominant polycystic kidney disease: the changing face of clinical management. The Lancet, 385(9981), 1993-2002.
Streets AJ, Wessely O, Peters DJM & Ong ACM (2013) Hyperphosphorylation of polycystin-2 at a critical residue in disease reveals an essential role for polycystin-1-regulated dephosphorylation.. Hum Mol Genet, 22(10), 1924-1939.
Giamarchi A, Feng S, Rodat-Despoix L, Xu YX, Bubenshchikova E, Newby LJ, Hao JZ, Gaudioso C, Crest M, Lupas AN , Honore E et al (2010) A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes. EMBO J, 29(7), 1176-1191. View this article in WRRO
Ong ACM & Wheatley DN (2003) Polycystic kidney disease--the ciliary connection.. Lancet, 361(9359), 774-776.
Newby LJ, Streets AJ, Zhao Y, Harris PC, Ward CJ & Ong ACM (2002) Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex.. J Biol Chem, 277(23), 20763-20773. View this article in WRRO
Ong AC, Harris PC, Davies DR, Pritchard L, Rossetti S, Biddolph S, Vaux DJ, Migone N & Ward CJ (1999) Polycystin-1 expression in PKD1, early-onset PKD1, and TSC2/PKD1 cystic tissue.. Kidney Int, 56(4), 1324-1333.
Ong AC, Ward CJ, Butler RJ, Biddolph S, Bowker C, Torra R, Pei Y & Harris PC (1999) Coordinate expression of the autosomal dominant polycystic kidney disease proteins, polycystin-2 and polycystin-1, in normal and cystic tissue.. Am J Pathol, 154(6), 1721-1729.
Ward CJ, Turley H, Ong AC, Comley M, Biddolph S, Chetty R, Ratcliffe PJ, Gattner K & Harris PC (1996) Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney.. Proc Natl Acad Sci U S A, 93(4), 1524-1528.

All publications

Journal articles

Ong ACM & Gansevoort RT (2021) TAMEing ADPKD with metformin : safe and effective?. Kidney International, 100(3), 513-515.
Li D, Hu M, Chen H, Wu X, Wei X, Lin H, Gao X, Wang H, Li M, Ong ACM , Yue Z et al (2021) An Nphp1 knockout mouse model targeting exon 2–20 demonstrates characteristic phenotypes of human Nephronophthisis. Human Molecular Genetics.
Winterbottom J, Simms R, Caroli A, Cornec-Le Gall E, Demoulin N, Furlano M, Meijer E, Devuyst O, Gansevoort R, LeMeur Y , Perico N et al (2021) MO023FLANK PAIN HAS A MAJOR NEGATIVE IMPACT ON HEALTH-RELATED QUALITY OF LIFE IN ADPKD: THE CYSTIC I STUDY. Nephrology Dialysis Transplantation, 36(Supplement_1).
Mao Z, Valluru MK & Ong ACM (2021) Drug repurposing in autosomal dominant polycystic kidney disease: back to the future with pioglitazone. Clinical Kidney Journal. View this article in WRRO
Zhou S, Xu J, Xue C, Yang B, Mao Z & Ong ACM (2021) Coronavirus-associated kidney outcomes in COVID-19, SARS, and MERS: a meta-analysis and systematic review. Renal Failure, 43(1), 1-15.
Fragiadaki M, Macleod FM & Ong ACM (2020) The controversial role of fibrosis in Autosomal Dominant Polycystic Kidney Disease. International Journal of Molecular Sciences, 21(23). View this article in WRRO
Durkie M, Chong J, Valluru MK, Harris PC & Ong ACM (2020) Biallelic inheritance of hypomorphic PKD1 variants is highly prevalent in very early onset polycystic kidney disease. Genetics in Medicine.
Logeman C, Cho Y, Sautenet B, Rangan GK, Gutman T, Craig J, Ong A, Chapman A, Ahn C, Coolican H , Tze-Wah Kao J et al (2020) ‘A sword of Damocles’ : patient and caregiver beliefs, attitudes and perspectives on presymptomatic testing for autosomal dominant polycystic kidney disease: a focus group study. BMJ Open, 10(10).
Streets AJ, Prosseda PP & Ong ACM (2020) Polycystin-1 regulates ARHGAP35-dependent centrosomal RhoA activation and ROCK signaling. JCI Insight, 5(16).
Cho Y, Tong A, Craig JC, Mustafa RA, Chapman A, Perrone RD, Ahn C, Fowler K, Torres V, Gansevoort RT , Ong ACM et al (2020) Establishing a core outcome set for autosomal dominant polycystic kidney disease : report of the Standardized Outcomes in Nephrology–Polycystic Kidney Disease (SONG-PKD) consensus workshop. American Journal of Kidney Diseases.
Streets A & Ong A (2020) Post-translational modifications of the polycystin proteins. Cellular Signalling, 72.
Živná M, Kidd K, Zaidan M, Vyleťal P, Barešová V, Hodaňová K, Sovová J, Hartmannová H, Votruba M, Trešlová H , Jedličková I et al (2020) An international cohort study of autosomal dominant tubulointerstitial kidney disease due to REN mutations identifies distinct clinical subtypes. Kidney International. View this article in WRRO
Thaventhiran JED, Lango Allen H, Burren OS, Rae W, Greene D, Staples E, Zhang Z, Farmery JHR, Simeoni I, Rivers E , Maimaris J et al (2020) Publisher Correction: Whole-genome sequencing of a sporadic primary immunodeficiency cohort. Nature, 584(7819), E2-E2.
Torres VE & Ong ACM (2020) Cellular signaling in PKD: foreword. Cellular Signalling, 71. View this article in WRRO
Winterbottom J, Simms R & Ong A (2020) SO092REDUCED QUALITY OF LIFE IN ADPKD PATIENTS WITH CKD STAGE 1-3: THE CYSTIC I QUALITY OF LIFE STUDY. Nephrology Dialysis Transplantation, 35(Supplement_3).
Thaventhiran JED, Lango Allen H, Burren OS, Rae W, Greene D, Staples E, Zhang Z, Farmery JHR, Simeoni I, Rivers E , Maimaris J et al (2020) Whole-genome sequencing of a sporadic primary immunodeficiency cohort. Nature. View this article in WRRO
Cho Y, Rangan G, Logeman C, Ryu H, Sautenet B, Perrone RD, Nadeau-Fredette A-C, Mustafa RA, Htay H, Chonchol M , Harris T et al (2020) Core outcome domains for trials in autosomal dominant polycystic kidney disease: An international Delphi survey. American Journal of Kidney Diseases. View this article in WRRO
Huynh VT, Audrézet M-P, Sayer JA, Ong AC, Lefevre S, Le Brun V, Després A, Senum SR, Chebib F, Barroso-Gil M , Patel C et al (2020) Clinical spectrum, prognosis and estimated prevalence of DNAJB11-kidney disease. Kidney International. View this article in WRRO
Sautenet B, Cho Y, Gutman T, Rangan G, Ong A, Chapman AB, Ahn C, Coolican H, Tze-Wah Kao J, Fowler K , Gansevoort RT et al (2020) Range and variability of outcomes reported in randomized trials conducted in patients with polycystic kidney disease: A systematic review. American Journal of Kidney Diseases. View this article in WRRO
Lannoy M, Valluru MK, Chang L, Abdela-Ali F, Peters DJM, Streets AJ & Ong ACM (2020) The positive effect of selective prostaglandin E2 receptor EP2 and EP4 blockade on cystogenesis in vitro is counteracted by increased kidney inflammation in vivo. Kidney International. View this article in WRRO
Magayr TA, Song X, Streets AJ, Vergoz L, Chang L, Valluru MK, Yap HL, Lannoy M, Haghighi A, Simms RJ , Tam FWK et al (2020) Global microRNA profiling in human urinary exosomes reveals novel disease biomarkers and cellular pathways for Autosomal Dominant Polycystic Kidney Disease. Kidney International. View this article in WRRO
Griffiths J, Mills MT & Ong ACM (2020) Long-acting somatostatin analogue treatments in autosomal dominant polycystic kidney disease and polycystic liver disease : a systematic review and meta-analysis. BMJ Open, 10(1). View this article in WRRO
Metzner A, Griffiths JD, Streets AJ, Markham E, Philippou T, Van Eeden FJM & Ong ACM (2020) A high throughput zebrafish chemical screen reveals ALK5 and non-canonical androgen signalling as modulators of the pkd2−/− phenotype. Scientific Reports, 10(1). View this article in WRRO
Hopp K, Cornec-Le Gall E, Senum SR, te Paske IBAW, Raj S, Lavu S, Baheti S, Edwards ME, Madsen CD, Heyer CM , Ong ACM et al (2020) Detection and characterization of mosaicism in autosomal dominant polycystic kidney disease. Kidney International, 97(2), 370-382.
Annear NMP, Appleton RE, Bassi Z, Bhatt R, Bolton PF, Crawford P, Crowe A, Tossi M, Elmslie F, Finlay E , Gale DP et al (2019) Tuberous sclerosis complex (TSC) : expert recommendations for provision of coordinated care. Frontiers in Neurology, 10. View this article in WRRO
LOGEMAN C, Cho YJ, Sautenet B, Rangan G, Talia G, Johnathan C, Ong A, Arlene C, Curie A & Helen C (2019) SAT-100 “A SWORD OF DAMOCLES”: PATIENT AND CAREGIVER BELIEFS, ATTITUDES AND PERSPECTIVES ON GENETIC SCREENING AND TESTING FOR AUTOSOMAL POLYCYSTIC KIDNEY DISEASE - FOCUS GROUP STUDY. Kidney International Reports, 4(7), S48-S48.
Durkie M, Chong J & Ong A (2019) SAT-334 GENETIC TESTING OF FAMILIES WITH VERY EARLY ONSET POLYCYSTIC KIDNEY DISEASE REVEALS THE FUNCTIONAL SIGNIFICANCE OF HYPOMORPHIC VARIANTS. Kidney International Reports, 4(7), S148-S148.
Omar F, Findlay JE, Carfray G, Allcock RW, Jiang Z, Moore C, Muir AL, Lannoy M, Fertig BA, Mai D , Day JP et al (2019) Small-molecule allosteric activators of PDE4 long form cyclic AMP phosphodiesterases.. Proceedings of the National Academy of Sciences, 116(27), 13320-13329. View this article in WRRO
Huynh VT, Audrézet M-P, Domenger C, Bridoux F, Seret G, Longuet H, Halimi J-M, Genkyst SG, Senum S, Pajot A , Albaret J et al (2019) SaO005CLINICAL PRESENTATION AND PROGNOSIS OF DNAJB11-ASSOCIATED NEPHROPATHY: AN INTERNATIONAL COLLABORATIVE STUDY. Nephrology Dialysis Transplantation, 34(Supplement_1).
MAGAYR T, Streets A & Ong A (2019) FO026GLOBAL MICRORNA PROFILING IN HUMAN URINARY EXOSOMES REVEALS NEW DISEASE BIOMARKERS AND CELLULAR PATHWAYS FOR AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD). Nephrology Dialysis Transplantation, 34(Supplement_1).
Tam FWK & Ong ACM (2019) Renal monocyte chemoattractant protein-1: an emerging universal biomarker and therapeutic target for kidney diseases?. Nephrology,Dialysis and Transplantation. View this article in WRRO
Simms RJ, Doshi T, Metherall P, Ryan D, Wright P, Gruel N, van Gastel MDA, Gansevoort RT, Tindale W & Ong ACM (2019) A rapid high-performance semi-automated tool to measure total kidney volume from MRI in autosomal dominant polycystic kidney disease. European Radiology, 1-10. View this article in WRRO
Cho Y, Sautenet B, Gutman T, Rangan G, Craig JC, Ong AC, Chapman A, Ahn C, Coolican H, Kao JTW , Gansevoort R et al (2019) Identifying patient‐important outcomes in polycystic kidney disease: An international nominal group technique study. Nephrology. View this article in WRRO
Gimpel C, Avni EF, Breysem L, Burgmaier K, Caroli A, Cetiner M, Haffner D, Hartung EA, Franke D, König J , Liebau MC et al (2019) Imaging of Kidney Cysts and Cystic Kidney Diseases in Children: An International Working Group Consensus Statement.. Radiology. View this article in WRRO
Tong A, Manns B, Wang AYM, Hemmelgarn B, Wheeler DC, Gill J, Tugwell P, Pecoits-Filho R, Crowe S, Harris T , Van Biesen W et al (2018) Implementing core outcomes in kidney disease: report of the Standardized Outcomes in Nephrology (SONG) implementation workshop. Kidney International, 94(6), 1053-1068.
Selby NM, Blankestijn PJ, Boor P, Combe C, Eckardt K, Eikefjord E, Garcia-Fernandez N, Golay X, Gordon I, Grenier N , Hockings PD et al (2018) Magnetic resonance imaging biomarkers for chronic kidney disease: a position paper from the European Cooperation in Science and Technology Action PARENCHIMA. Nephrology Dialysis Transplantation, 33(Sup 2), ii4-ii14. View this article in WRRO
Harris T, Sandford R, De Coninck B, Devuyst O, Drenth JPH, Ecder T, Kent A, Gansevoort RT, Górriz JL, Ong ACM , Pirson Y et al (2018) European ADPKD Forum multidisciplinary position statement on autosomal dominant polycystic kidney disease care: European ADPKD Forum and Multispecialist Roundtable participants. Nephrology Dialysis Transplantation, 33(4), 563-573. View this article in WRRO
Neagu M, Coca D & Ong ACM (2018) Linear and Nonlinear Estimated GFR Slopes in ADPKD Patients Reaching ESRD.. Am J Kidney Dis. View this article in WRRO
McEwan P, Bennett Wilton H, Ong ACM, Ørskov B, Sandford R, Scolari F, Cabrera M-CV, Walz G, O’Reilly K & Robinson P (2018) A model to predict disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD): the ADPKD Outcomes Model. BMC Nephrology, 19(1). View this article in WRRO
Ong ACM (2018) Tolvaptan slows disease progression in late-stage ADPKD. Nature Reviews Nephrology, 14(3), 146-148.
Chang M-Y & Ong ACM (2017) Targeting new cellular disease pathways in autosomal dominant polycystic kidney disease. Nephrology Dialysis Transplantation, 32(12), 2144-2144.
Cho Y, Sautenet B, Rangan G, Craig JC, Ong ACM, Chapman A, Ahn C, Chen D, Coolican H, Kao JT-W , Gansevoort R et al (2017) Standardised Outcomes in Nephrology—Polycystic Kidney Disease (SONG-PKD): study protocol for establishing a core outcome set in polycystic kidney disease. Trials, 18(1). View this article in WRRO
Feng S, Streets AJ, Nesin V, Tran U, Nie H, Onopiuk M, Wessely O, Tsiokas L & Ong A (2017) The Sorting Nexin 3 Retromer Pathway Regulates the Cell Surface Localization and Activity of a Wnt-Activated Polycystin Channel Complex. Journal of the American Society of Nephrology : JASN, 28(10), 2973-2984. View this article in WRRO
Chang M-Y & C.M. Ong A (2017) Targeting new cellular disease pathways in autosomal dominant polycystic kidney disease. Nephrology Dialysis Transplantation.
Ong ACM (2017) Making sense of polycystic kidney disease. The Lancet, 389(10081), 1780-1782.
Chong J, Durkie M, Dalton A & Ong A (2017) TO033VERY EARLY-ONSET AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE DUE TO BI-ALLELIC MUTATIONS IN PKD1 AND PKD2. Nephrology Dialysis Transplantation, 32(suppl_3), iii94-iii94.
Streets AJ, Magayr TA, Huang L, Vergoz L, Rossetti S, Simms RJ, Harris PC, Peters DJM & Ong ACM (2017) Parallel microarray profiling identifies ErbB4 as a determinant of cyst growth in ADPKD and a prognostic biomarker for disease progression. American Journal of Physiology - Renal Physiology, 312(4), F577-F588. View this article in WRRO
Fragiadaki M, Lannoy M, Themanns M, Maurer B, Leonhard WN, Peters DJM, Moriggl R & Ong ACM (2017) STAT5 drives abnormal proliferation in autosomal dominant polycystic kidney disease. Kidney International, 91(3), 575-586. View this article in WRRO
Simms R, Ryan D, Metherall P, Wright P, Gruel N, Tindale W & Ong A (2017) Development of a rapid semi-automated tool to measure total kidney volume in autosomal dominant polycystic kidney disease. The Lancet, 389, S90-S90. View this article in WRRO
Mao Z, Chong J & Ong ACM (2016) Autosomal dominant polycystic kidney disease: recent advances in clinical management. F1000Research, 5, 2029-2029. View this article in WRRO
Lannoy M, Abdela-ali F, Streets AJ & Ong ACM (2016) SO053SELECTIVE PROSTAGLANDIN E2 RECEPTOR BLOCKADE FOR THE TREATMENT OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE. Nephrology Dialysis Transplantation, 31(suppl_1), i22-i23.
Gansevoort RT, Arici M, Benzing T, Birn H, Capasso G, Covic A, Devuyst O, Drechsler C, Eckardt K-U, Emma F , Knebelmann B et al (2016) Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice. Nephrology Dialysis Transplantation, 31(3), 337-348. View this article in WRRO
Xu Y, Streets AJ, Hounslow AM, Tran U, Jean-Alphonse F, Needham AJ, Vilardaga J-P, Wessely O, Williamson MP & Ong ACM (2016) The Polycystin-1, Lipoxygenase, and α-Toxin Domain Regulates Polycystin-1 Trafficking. Journal of the American Society of Nephrology, 27(4), 1159-1173.
Simms RJ, Thong KM, Dworschak GC & Ong ACM (2016) Increased psychosocial risk, depression and reduced quality of life living with autosomal dominant polycystic kidney disease. Nephrology Dialysis Transplantation, 31(7), 1130-1140.
Yang B, Chen S, Yang G, Mei C, Ong A & Mao Z (2015) New onset diabetes after kidney transplantation in patients with autosomal dominant polycystic kidney disease: systematic review protocol: Figure 1. BMJ Open, 5(11). View this article in WRRO
Ong ACM & Harris PC (2015) A polycystin-centric view of cyst formation and disease: the polycystins revisited. Kidney International, 88(4), 699-710. View this article in WRRO
Cantero MDR, Velázquez IF, Streets AJ, Ong ACM & Cantiello HF (2015) The cAMP Signaling Pathway and Direct Protein Kinase A Phosphorylation Regulate Polycystin-2 (TRPP2) Channel Function. Journal of Biological Chemistry, 290(39), 23888-23896.
Chapman AB, Devuyst O, Eckardt K-U, Gansevoort RT, Harris T, Horie S, Kasiske BL, Odland D, Pei Y, Perrone RD , Pirson Y et al (2015) Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney International, 88(1), 17-27.
(2015) Department of Error. The Lancet, 385(9987), 2576-2576.
Simms RJ, Thong KM, Dworschak GC & Ong ACM (2015) FP364INCREASED PSYCHOSOCIAL BURDEN AND ADVERSE QUALITY OF LIFE IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE. Nephrology Dialysis Transplantation, 30(suppl_3), iii190-iii190.
Ong ACM, Devuyst O, Knebelmann B & Walz G (2015) Autosomal dominant polycystic kidney disease: the changing face of clinical management. The Lancet, 385(9981), 1993-2002.
Xu Y, Ong ACM, Williamson MP & Hounslow AM (2015) Backbone assignment and secondary structure of the PLAT domain of human polycystin-1. Biomolecular NMR Assignments, 9(2), 369-373.
Streets AJ, Xu Y & Ong ACM (2015) SuO042POLYCYSTIN-1 TRAFFICKING IS REGULATED BY CAMP DEPENDENT PHOSPHORYLATION OF THE PLAT DOMAIN. Nephrology Dialysis Transplantation, 30(suppl_3), iii63-iii63.
Mao Z, Xie G & Ong ACM (2015) Metabolic abnormalities in autosomal dominant polycystic kidney disease. Nephrology Dialysis Transplantation, 30(2), 197-203.
Ong ACM, von Websky K & Hocher B (2015) Endothelin and Tubulointerstitial Renal Disease. Seminars in Nephrology, 35(2), 197-207.
Simms RJ, Travis DL, Durkie M, Wilson G, Dalton A & Ong ACM (2015) Genetic Testing in the Assessment of Living Related Kidney Donors at Risk of Autosomal Dominant Polycystic Kidney Disease. Transplantation, 99(5), 1023-1029.
McEwan P, Bennett Wilton H, Robinson P, Hadimeri H, Ong A, Ørskov B, Peces R, Sandford R, Scolari F, Walz G , Woon C et al (2014) Development Of A Model To Predict Disease Progression In Autosomal Dominant Polycystic Kidney Disease (ADPKD). Value in Health, 17(7), A564-A564.
Petzold K, Gansevoort RT, Ong ACM, Devuyst O, Rotar L, Eckardt K-U, Köttgen A, Pirson Y, Remuzzi G, Sandford R , Tesar V et al (2014) Building a network of ADPKD reference centres across Europe: the EuroCYST initiative. Nephrology Dialysis Transplantation, 29(suppl_4), iv26-iv32.
Thong KM & Ong ACM (2014) Sudden death due to subarachnoid haemorrhage in an infant with autosomal dominant polycystic kidney disease. Nephrology Dialysis Transplantation, 29(suppl 4), iv121-iv123.
Simms RJ & Ong ACM (2014) How simple are 'simple renal cysts'?. Nephrology Dialysis Transplantation, 29(suppl 4), iv106-iv112.
Devuyst O, Knoers NVAM, Remuzzi G & Schaefer F (2014) Rare inherited kidney diseases: challenges, opportunities, and perspectives. The Lancet, 383(9931), 1844-1859.
Cabrera-López C, Ars E, Marti T, Harris PC, Torra R, Clerckx C, Migeon T, Chen Z, Ronco P, Plaisier E , Lamers IJ et al (2014) Renal development and cystic diseases.. Nephrol Dial Transplant, 29 Suppl 3, iii73-iii78.
Alfieri A, Ong ACM, Kammerer RA, Solanky T, Bate S, Tasab M, Brown NJ & Brookes ZL (2014) Angiopoietin-1 regulates microvascular reactivity and protects the microcirculation during acute endothelial dysfunction: role of eNOS and VE-cadherin.. Pharmacol Res, 80, 43-51.
(2014) Erratum. Nephrology Dialysis Transplantation, 29(12), 2353-2353.
Spithoven EM, Kramer A, Meijer E, Orskov B, Wanner C, Abad JM, Areste N, Alonso de la Torre R, Caskey F, Couchoud C , Finne P et al (2014) Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe: prevalence and survival--an analysis of data from the ERA-EDTA Registry. Nephrology Dialysis Transplantation, 29(suppl 4), iv15-iv25.
Spithoven EM, Kramer A, Meijer E, Orskov B, Wanner C, Caskey F, Collart F, Finne P, Fogarty DG, Groothoff JW , Hoitsma A et al (2014) Analysis of data from the ERA-EDTA Registry indicates that conventional treatments for chronic kidney disease do not reduce the need for renal replacement therapy in autosomal dominant polycystic kidney disease. Kidney International, 86(6), 1244-1252.
Lin L, Xu Y, Zhang S, Ross IM, Ong ACM & Allwood DA (2014) Fabrication and luminescence of monolayered boron nitride quantum dots.. Small, 10(1), 60-65.
Chang MY & Ong ACM (2013) New treatments for autosomal dominant polycystic kidney disease. British Journal of Clinical Pharmacology, 76(4), 524-535.
Thong KM, Xu Y, Cook J, Takou A, Wagner B, Kawar B & Ong ACM (2013) Cosegregation of focal segmental glomerulosclerosis in a family with familial partial lipodystrophy due to a mutation in LMNA.. Nephron Clin Pract, 124(1-2), 31-37.
Lin L, Xu Y, Zhang S, Ross IM, Ong ACM & Allwood DA (2013) Fabrication of luminescent monolayered tungsten dichalcogenides quantum dots with giant spin-valley coupling.. ACS Nano, 7(9), 8214-8223.
Liu Y, Käch A, Ziegler U, Ong ACM, Wallace DP, Arcaro A & Serra AL (2013) The Role of Phospholipase D in Modulating the MTOR Signaling Pathway in Polycystic Kidney Disease. PLoS ONE, 8(8). View this article in WRRO
Ong ACM (2013) Primary cilia and renal cysts: does length matter?. Nephrol Dial Transplant, 28(11), 2661-2663.
Thong KM & Ong ACM (2013) The natural history of autosomal dominant polycystic kidney disease: 30-year experience from a single centre.. QJM, 106(7), 639-646.
Streets AJ, Wessely O, Peters DJM & Ong ACM (2013) Hyperphosphorylation of polycystin-2 at a critical residue in disease reveals an essential role for polycystin-1-regulated dephosphorylation.. Hum Mol Genet, 22(10), 1924-1939.
Mekahli D, Decuypere J-P, Sammels E, Welkenhuyzen K, Bultynck G, Parys JB, Missiaen L, De Smedt H, Levtchenko E, Schoeber J , van den Heuvel L et al (2013) Polycystin-1 but not polycystin-2 deficiency causes upregulation of the mTOR pathway and can be synergistically targeted with rapamycin and metformin. Pflugers Archiv European Journal of Physiology, 1-14.
Brookes ZLS, Ruff L, Upadhyay VS, Huang L, Prasad S, Solanky T, Nauli SM & Ong ACM (2013) Pkd2 mesenteric vessels exhibit a primary defect in endothelium-dependent vasodilatation restored by rosiglitazone.. Am J Physiol Heart Circ Physiol, 304(1), H33-H41.
Loftus H & Ong ACM (2013) Cystic kidney diseases: many ways to form a cyst.. Pediatr Nephrol, 28(1), 33-49. View this article in WRRO
Devuyst O, Antignac C, Bindels RJM, Chauveau D, Emma F, Gansevoort R, Maxwell PH, Ong ACM, Remuzzi G, Ronco P & Schaefer F (2012) The ERA-EDTA Working Group on inherited kidney disorders. Nephrology Dialysis Transplantation, 27(1), 67-69.
Chang M-Y & Ong ACM (2012) Mechanism-Based Therapeutics for Autosomal Dominant Polycystic Kidney Disease: Recent Progress and Future Prospects. NEPHRON CLINICAL PRACTICE, 120(1), C25-C34.
Mao Z & Ong ACM (2012) Spurious hypophosphatemia associated with monoclonal paraproteinemia.. QJM, 105(7), 693-696.
Mekahli D, Sammels E, Decuypere JP, Audrezet MP, Dechenes G, Ong A, van den Heuvel L, Missiaen L, De Smedt H & Levtchenko E (2011) mTOR signaling is differentially affected in human renal cells with PKD1 or PKD2 mutations. PEDIATR NEPHROL, 26(9), 1648-1648.
Feng S, Rodat-Despoix L, Delmas P & Ong ACM (2011) A single amino acid residue constitutes the third dimerization domain essential for the assembly and function of the tetrameric polycystin-2 (TRPP2) channel.. J Biol Chem, 286(21), 18994-19000. View this article in WRRO
Mao Z, Streets AJ & Ong ACM (2011) Thiazolidinediones inhibit MDCK cyst growth through disrupting oriented cell division and apicobasal polarity.. Am J Physiol Renal Physiol, 300(6), F1375-F1384.
Ong ACM & Devuyst O (2011) Towards the Integration of Genetic Knowledge into Clinical Practice. NEPHRON CLIN PRACT, 118(1), C3-C8.
Chang M-Y & Ong ACM (2011) Endothelin in polycystic kidney disease.. Contrib Nephrol, 172, 200-209.
Ong ACM & Gubler MC (2011) What's new in... Ciliopathies. Medicine, 39(2), 119-125.
Streets AJ, Needham AJ, Gill SK & Ong ACM (2010) Protein kinase D-mediated phosphorylation of polycystin-2 (TRPP2) is essential for its effects on cell growth and calcium channel activity.. Mol Biol Cell, 21(22), 3853-3865.
Giamarchi A, Feng S, Rodat-Despoix L, Xu YX, Bubenshchikova E, Newby LJ, Hao JZ, Gaudioso C, Crest M, Lupas AN , Honore E et al (2010) A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes. EMBO J, 29(7), 1176-1191. View this article in WRRO
Yu Y, Ulbrich MH, Li M-H, Buraei Z, Chen X-Z, Ong ACM, Tong L, Isacoff EY & Yang J (2010) Structural and Molecular Basis of the Assembly of the TRPP2/PKD1 Complex. Biophysical Journal, 98(3), 344a-344a.
Yu Y, Ulbrich MH, Li M-H, Buraei Z, Chen X-Z, Ong ACM, Tong L, Isacoff EY & Yang J (2010) Structural and Molecular Basis of the Assembly of the TRPP2/PKD1 Complex. BIOPHYSICAL JOURNAL, 98(3), 343A-343A.
(2009) Screening for intracranial aneurysms in ADPKD. BMJ, 339(oct13 2), b4204-b4204.
Prasad S, McDaid JP, Tam FWK, Haylor JL & Ong ACM (2009) Pkd2 dosage influences cellular repair responses following ischemia-reperfusion injury.. Am J Pathol, 175(4), 1493-1503. View this article in WRRO
Ong ACM (2009) Screening for intracranial aneurysms in ADPKD.. BMJ, 339, b3763. View this article in WRRO
Laycock S, Taylor HC, Haigh C, Lee AT, Cooper GJ, Ong ACM & Robson L (2009) A novel dephosphorylation-activated conductance in a mouse renal collecting duct cell line.. Exp Physiol, 94(8), 914-927.
Chow CL & Ong ACM (2009) Autosomal dominant polycystic kidney disease.. Clin Med (Lond), 9(3), 278-283.
Streets AJ, Wagner BE, Harris PC, Ward CJ & Ong ACM (2009) Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells.. J Cell Sci, 122(Pt 9), 1410-1417. View this article in WRRO
Mao Z & Ong ACM (2009) Peroxisome proliferator-activated receptor gamma agonists in kidney disease--future promise, present fears.. Nephron Clin Pract, 112(4), c230-c241. View this article in WRRO
Streets AJ, Wagner BE, Harris PC, Ward CJ & Ong ACM (2009) Erratum: Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells (Journal of Cell Science vol. 122 (1410-1417)). Journal of Cell Science, 122(10), 1702.
Yu Y, Ulbrich MH, Li MH, Buraei Z, Chen XZ, Ong ACM, Tong L, Isacoff EY & Yang J (2009) Structural and molecular basis of the assembly of the TRPP2/PKD1 complex. Proceedings of the National Academy of Sciences of the United States of America, 106(28), 11558-11563. View this article in WRRO
Feng S, Okenka GM, Bai C-X, Streets AJ, Newby LJ, DeChant BT, Tsiokas L, Obara T & Ong ACM (2008) Identification and functional characterization of an N-terminal oligomerization domain for polycystin-2.. J Biol Chem, 283(42), 28471-28479. View this article in WRRO
Bai CX, Kim S, Li WP, Streets AJ, Ong AC & Tsiokas L (2008) Activation of TRPP2 through mDia1-dependent voltage gating. EMBO J, 27(9), 1345-1356.
Chang M-Y & Ong ACM (2008) Autosomal dominant polycystic kidney disease: recent advances in pathogenesis and treatment.. Nephron Physiol, 108(1), p1-p7.
Parker E, Newby LJ, Sharpe CC, Rossetti S, Streets AJ, Harris PC, O'Hare MJ & Ong ACM (2007) Hyperproliferation of PKD1 cystic cells is induced by insulin-like growth factor-1 activation of the Ras/Raf signalling system.. Kidney Int, 72(2), 157-165.
Chang M-Y, Parker E, El Nahas M, Haylor JL & Ong ACM (2007) Endothelin B receptor blockade accelerates disease progression in a murine model of autosomal dominant polycystic kidney disease.. J Am Soc Nephrol, 18(2), 560-569.
Hamer RA, Chow CL, Ong ACM & McKane WS (2007) Polycystic kidney disease is a risk factor for new-onset diabetes after transplantation.. Transplantation, 83(1), 36-40.
Bernhardt WM, Wiesener MS, Weidemann A, Schmitt R, Weichert W, Lechler P, Campean V, Ong ACM, Willam C, Gretz N & Eckardt KU (2007) Involvement of hypoxia-inducible transcription factors in polycystic kidney disease. American Journal of Pathology, 170(3), 830-842.
Ali A, Christie PT, Grigorieva IV, Harding B, Van Esch H, Ahmed SF, Bitner-Glindzicz M, Blind E, Bloch C, Christin P , Clayton P et al (2007) Functional characterization of GATA3 mutations causing the hypoparathyroidism-deafness-renal (HDR) dysplasia syndrome: Insight into mechanisms of DNA binding by the GATA3 transcription factor. Human Molecular Genetics, 16(3), 265-275.
Chang MY, Parker E, Ibrahim S, Shortland JR, Nahas ME, Haylor JL & Ong ACM (2006) Haploinsufficiency of Pkd2 is associated with increased tubular cell proliferation and interstitial fibrosis in two murine Pkd2 models.. Nephrol Dial Transplant, 21(8), 2078-2084.
Streets AJ, Moon DJ, Kane ME, Obara T & Ong ACM (2006) Identification of an N-terminal glycogen synthase kinase 3 phosphorylation site which regulates the functional localization of polycystin-2 in vivo and in vitro.. Hum Mol Genet, 15(9), 1465-1473. View this article in WRRO
Ong ACM & Wagner B (2005) Detection of proximal tubular motile cilia in a patient with renal sarcoidosis associated with hypercalcemia.. Am J Kidney Dis, 45(6), 1096-1099.
Ong ACM & Harris PC (2005) Molecular pathogenesis of ADPKD: the polycystin complex gets complex.. Kidney Int, 67(4), 1234-1247.
Le NH, van der Bent P, Huls G, van de Wetering M, Loghman-Adham M, Ong ACM, Calvet JP, Clevers H, Breuning MH, van Dam H & Peters DJM (2004) Aberrant polycystin-1 expression results in modification of activator protein-1 activity, whereas Wnt signaling remains unaffected. J BIOL CHEM, 279(26), 27472-27481. View this article in WRRO
Streets AJ, Newby LJ, O'Hare MJ, Bukanov NO, Ibraghimov-Beskrovnaya O & Ong ACM (2003) Functional analysis of PKD1 transgenic lines reveals a direct role for polycystin-1 in mediating cell-cell adhesion.. J Am Soc Nephrol, 14(7), 1804-1815.
Ong ACM (2003) Genetic Renal Abnormalities. Medicine, 31(5), 32-35.
Ong ACM & Wheatley DN (2003) Polycystic kidney disease--the ciliary connection.. Lancet, 361(9359), 774-776.
Ong ACM, Newby LJ & Dashwood MR (2003) Expression and cellular localisation of renal endothelin-1 and endothelin receptor subtypes in autosomal-dominant polycystic kidney disease. NEPHRON EXP NEPHROL, 93(2), E80-E86.
Rossetti S, Chauveau D, Kubly V, Slezak JM, Saggar-Malik AK, Pei Y, Ong ACM, Stewart F, Watson ML, Bergstralh EJ , Winearls CG et al (2003) Association of mutation position in polycystic kidney disease 1 (PKD1) gene and development of a vascular phenotype. Lancet, 361(9376), 2196-2201.
Ong ACM, Newby LJ & Dashwood MR (2003) Expression and cellular localisation of renal endothelin-1 and endothelin receptor subtypes in autosomal-dominant polycystic kidney disease.. Nephron Exp Nephrol, 93(2), e80.
Zhao Y, Haylor JL & Ong ACM (2002) Polycystin-2 expression is increased following experimental ischaemic renal injury.. Nephrol Dial Transplant, 17(12), 2138-2144.
Harris P, Rossetti S, Chauveau D, Kubly V, Bergstralh E, Slezak J, Ong A, Sagger-Malik A, Pei Y, Winearls C & Torres V (2002) Genotype/phenotype correlations in ADPKD: Vascular complications.. J AM SOC NEPHROL, 13, 40A-40A.
Streets AJ, Ibraghimov-Beskrovnaya O & Ong ACM (2002) Polycystin-1 mediates cell-cell adhesion in PKD1 transgenic and non-transgenic lines. J AM SOC NEPHROL, 13, 46A-46A.
Newby LJ, Streets AJ, Zhao Y, Harris PC, Ward CJ & Ong ACM (2002) Identification, characterization, and localization of a novel kidney polycystin-1-polycystin-2 complex.. J Biol Chem, 277(23), 20763-20773. View this article in WRRO
Ong ACM (2000) Polycystin expression in the kidney and other tissues: Complexity, consensus and controversy. EXP NEPHROL, 8(4-5), 208-214.
Ong AC (2000) Polycystin expression in the kidney and other tissues: complexity, consensus and controversy.. Exp Nephrol, 8(4-5), 208-214.
Benigni A, Madias NE, Remuzzi G, Siragy HM, Brooks DP, Rossi GP, Abbate M, Ong A, Loeffler BM, Bender N , Noris M et al (2000) Endothelin antagonists in renal disease. KIDNEY INT, 57(4), 1778-1794.
Ong AC, Harris PC, Davies DR, Pritchard L, Rossetti S, Biddolph S, Vaux DJ, Migone N & Ward CJ (1999) Polycystin-1 expression in PKD1, early-onset PKD1, and TSC2/PKD1 cystic tissue.. Kidney Int, 56(4), 1324-1333.
Ong ACM, Harris PC, Rossetti S, Davies DR & Ward CJ (1999) Polycystin expression in PKD-1, infantile PKD-1 and TSC-2/PKD-1 cystic tissue: heterogenous expression of the normal allele in cystic epithelia.. KIDNEY INT, 55(6), 2559-2559.
Ong AC, Ward CJ, Butler RJ, Biddolph S, Bowker C, Torra R, Pei Y & Harris PC (1999) Coordinate expression of the autosomal dominant polycystic kidney disease proteins, polycystin-2 and polycystin-1, in normal and cystic tissue.. Am J Pathol, 154(6), 1721-1729.
Ong AC, Harris PC, Biddolph S, Bowker C & Ward CJ (1999) Characterisation and expression of the PKD-1 protein, polycystin, in renal and extrarenal tissues.. Kidney Int, 55(5), 2091-2116.
Ong ACM, Harris PC, Biddolph S, Bowker C & Ward CJ (1999) Characterisation and expression of the PKD-1 protein, polycystin, in renal and extrarenal tissues.. KIDNEY INT, 55(5), 2091-2091.
Ong ACM, Mole DR & Altmann P (1999) An unusual complication of pregnancy. NEPHROL DIAL TRANSPL, 14(5), 1324-1326.
Ong AC, Mole DR & Altmann P (1999) An unusual complication of pregnancy.. Nephrol Dial Transplant, 14(5), 1324-1326.
Ong ACM (1999) Cyst formation in ADPKD: new insights from natural and targeted mutants. NEPHROL DIAL TRANSPL, 14(3), 544-546.
Ong AC (1999) Cyst formation in ADPKD: new insights from natural and targeted mutants.. Nephrol Dial Transplant, 14(3), 544-546.
Dashwood MR, Timm M, Muddle JR, Ong AC, Tippins JR, Parker R, McManus D, Murday AJ, Madden BP & Kaski JC (1998) Regional variations in endothelin-1 and its receptor subtypes in human coronary vasculature: pathophysiological implications in coronary disease.. Endothelium, 6(1), 61-70.
Ong ACM, Harris PC, Biddolph S, Bowker C & Ward CJ (1997) Characterisation, cellular expression, tissue localisation and developmental modulation of the PKD-1 product, polycystin.. J AM SOC NEPHROL, 8, A1744-A1744.
Ong ACM, Ward CJ, Biddolph S, Migone N & Harris PC (1997) Polycystin expression in PKD-1, infantile PKD-1 and TSC-2/PKD-1 cystic kidney: Evidence against a two-hit disease mechanism in cyst initiation.. J AM SOC NEPHROL, 8, A1745-A1745.
Peral B, Gamble V, Strong C, Ong AC, Sloane-Stanley J, Zerres K, Winearls CG & Harris PC (1997) Identification of mutations in the duplicated region of the polycystic kidney disease 1 gene (PKD1) by a novel approach.. Am J Hum Genet, 60(6), 1399-1410.
Ong AC & Harris PC (1997) Molecular basis of renal cyst formation--one hit or two?. Lancet, 349(9058), 1039-1040.
Ong AC (1997) Renal disease. II. The polycystic kidney disease 1 (PKD-1) gene: an important clue in the study of renal cyst formation.. J R Coll Physicians Lond, 31(2), 141-146.
Ong ACM (1997) The polycystic kidney disease 1 (PKD-1) gene: An important clue in the study of renal cyst formation. Journal of the Royal College of Physicians of London, 31(2), 141-146.
Peral B, Gamble V, Ong ACM, SanMillan JL & Harris PC (1996) Mutation defection in polycystic kidney disease 1 (PKD1) gene.. J AM SOC NEPHROL, 7(9), A1856-A1856.
Peral B, Ong AC, San Millán JL, Gamble V, Rees L & Harris PC (1996) A stable, nonsense mutation associated with a case of infantile onset polycystic kidney disease 1 (PKD1).. Hum Mol Genet, 5(4), 539-542.
Ward CJ, Turley H, Ong AC, Comley M, Biddolph S, Chetty R, Ratcliffe PJ, Gattner K & Harris PC (1996) Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney.. Proc Natl Acad Sci U S A, 93(4), 1524-1528.
Rowe PS, Ong AC, Cockerill FJ, Goulding JN & Hewison M (1996) Candidate 56 and 58 kDa protein(s) responsible for mediating the renal defects in oncogenic hypophosphatemic osteomalacia.. Bone, 18(2), 159-169.
Ong AC (1996) Perspectives in endothelin research: tubulointerstitial actions of endothelins in the kidney: roles in health and disease.. Nephrol Dial Transplant, 11(2), 251-257.
Ong ACM (1996) Surprising new roles for endothelins - Mutant mice reveal the first clear links to human disease. BRIT MED J, 312(7025), 195-196.
Ong AC (1996) Surprising new roles for endothelins.. BMJ, 312(7025), 195-196.
Peral B, SanMillan JL, Ong ACM, Gamble V, Ward CJ, Strong C & Harris PC (1996) Screening the 3' region of the polycystic kidney disease 1 (PKD1) gene reveals six novel mutations. AM J HUM GENET, 58(1), 86-96.
Ong ACM (1996) Surprising new roles for endothelins - Mutant mice reveal the first clear links to human disease. British Medical Journal, 312(7025), 195-196.
Ong ACM (1996) Tubulointerstitial actions of endothelins in the kidney: Roles in health and disease. Nephrology Dialysis Transplantation, 11(2), 251-257.
Peral B, San Millán JL, Ong AC, Gamble V, Ward CJ, Strong C & Harris PC (1996) Screening the 3' region of the polycystic kidney disease 1 (PKD1) gene reveals six novel mutations.. Am J Hum Genet, 58(1), 86-96.
WARD CJ, TURLEY H, ONG ACM, GATTER KC & HARRIS PC (1995) CHARACTERIZATION OF THE POLYCYSTIC-KIDNEY-DISEASE-1 (PKD1) PROTEIN, POLYCYSTIN. J AM SOC NEPHROL, 6(3), 713-713.
ONG ACM (1995) ENDOTHELIN DEPENDENT AND INDEPENDENT GROWTH SYSTEMS INVOLVED IN HUMAN PROXIMAL TUBULAR AUTOREGENERATION. J AM SOC NEPHROL, 6(3), 773-773.
Ong AC, Jowett TP, Firth JD, Burton S, Karet FE & Fine LG (1995) An endothelin-1 mediated autocrine growth loop involved in human renal tubular regeneration.. Kidney Int, 48(2), 390-401.
Rowe PSN, Hewison M, Cockerill FJ, Goulding JN, Ong A & O'Riordan JLH (1995) Oncogenous hypophosphataemic osteomalacia: effects on phosphate transport and vitamin D metabolism in cultured human kidney cells. Bone, 16(6), 679-679.
ONG ACM, FIRTH ID, IOWETT TP, BURTON S, KARET FE & FINE LG (1995) A HYPOXIA-INDUCIBLE, ENDOTHELIN-1 MEDIATED, AUTOCRINE GROWTH LOOP INVOLVED IN HUMAN RENAL TUBULAR REGENERATION. KIDNEY INT, 47(2), 693-693.
Fine LG, Norman JT & Ong A (1995) Cell-cell cross-talk in the pathogenesis of renal interstitial fibrosis. Kidney International, Supplement(49).
Ong AC, Jowett TP, Moorhead JF & Owen JS (1994) Human high density lipoproteins stimulate endothelin-1 release by cultured human renal proximal tubular cells.. Kidney Int, 46(5), 1315-1321.
ONG ACM, FIRTH JD, JOWETT TP, BURTON S, KARET FE & FINE LG (1994) AUTOREGENERATION OF HUMAN PROXIMAL TUBULAR CELLS MEDIATED BY A HYPOXIA-INDUCIBLE ENDOTHELIN-1 (ET-1) GROWTH LOOP. J AM SOC NEPHROL, 5(3), 699-699.
ONG ACM, JOWETT TP, FIRTH JD, BURTON S, KITAMURA M & FINE LG (1994) HUMAN TUBULAR-DERIVED ENDOTHELIN IN THE PARACRINE REGULATION OF RENAL INTERSTITIAL FIBROBLAST FUNCTION. EXP NEPHROL, 2(2), 134-134.
Ong AC & Moorhead JF (1994) Tubular lipidosis: epiphenomenon or pathogenetic lesion in human renal disease?. Kidney Int, 45(3), 753-762.
Ong AC, Jowett TP, Firth JD, Burton S, Kitamura M & Fine LG (1994) Human tubular-derived endothelin in the paracrine regulation of renal interstitial fibroblast function.. Exp Nephrol, 2(2), 134.
Ong AC & Fine LG (1994) Loss of glomerular function and tubulointerstitial fibrosis: cause or effect?. Kidney Int, 45(2), 345-351.
Ong AC & Fine LG (1994) Tubular-derived growth factors and cytokines in the pathogenesis of tubulointerstitial fibrosis: implications for human renal disease progression.. Am J Kidney Dis, 23(2), 205-209.
Ong AC & Fine LG (1994) Tubular-derived cytokines and human renal disease progression.. Nephrol Dial Transplant, 9(5), 471-472.
Fine LG, Ong AC & Norman JT (1993) Mechanisms of tubulo-interstitial injury in progressive renal diseases.. Eur J Clin Invest, 23(5), 259-265.
Ong AC, Jowett TP, Scoble JE, O'Shea JA, Varghese Z & Moorhead JF (1993) Effect of cyclosporin A on endothelin synthesis by cultured human renal cortical epithelial cells.. Nephrol Dial Transplant, 8(8), 748-753.
MACKIE ADR, SCOBLE J, ONG A, MILLWARD BA & DEMAINE AG (1992) ISOLATION OF THE NA+/H+ ANTIPORTER GENE EXPRESSED IN HUMAN PROXIMAL TUBULE CELLS. DIABETOLOGIA, 35, A56-A56.
ONG ACM, SCOBLE JE, BAILLOD RA, FERNANDO ON, SWENY P & MOORHEAD JF (1992) TUBERCULOUS PERITONITIS COMPLICATING PERITONEAL-DIALYSIS - A CASE FOR EARLY DIAGNOSTIC LAPAROTOMY. NEPHROL DIAL TRANSPL, 7(5), 443-446.
Ong AC, Scoble JE, Baillod RA, Fernando ON, Sweny P & Moorhead JF (1992) Tuberculous peritonitis complicating peritoneal dialysis: a case for early diagnostic laparotomy?. Nephrol Dial Transplant, 7(5), 443-446.
Ong AC & Handler CE (1991) Sinus arrest and asystole due to severe lithium intoxication.. Int J Cardiol, 30(3), 364-366.
Ong AC, Eisen V, Rennie DP, Homburg R, Lachelin GC, Jacobs HS & Slater JD (1991) The pathogenesis of the ovarian hyperstimulation syndrome (OHS): a possible role for ovarian renin.. Clin Endocrinol (Oxf), 34(1), 43-49.
TOMLINSON B, ONG ACM, GRAHAM BR & PRICHARD BNC (1990) EFFECTS OF NORADRENALINE INFUSION ON CARDIAC-OUTPUT AND SYSTOLIC-TIME INTERVALS DERIVED FROM ELECTRICAL BIOIMPEDANCE CARDIOGRAPHY. BRIT J CLIN PHARMACO, 29(5), P639-P640.
Ong AC, Handler CE & Slater JD (1990) Atrial natriuretic peptide release responds to atrial stretch and not to atrial pressure: observations during pericardiocentesis in a young woman.. Eur Heart J, 11(4), 368-371.
Tomlinson B, Ong ACM, Graham BR & Prichard BNC (1990) Effects of noradrenaline infusion on cardiac output and systolic time intervals derived from electrical bioimpedance cardiography. British Journal of Clinical Pharmacology, 29(5).
Ong AC (1990) The use of lithium clearance in Bartter's syndrome.. Nephrol Dial Transplant, 5(10), 904-905.
Tomlinson B, Smith CC, Ong AC, Graham BR, Betteridge DJ & Prichard BN (1989) Effects of noradrenaline infusion on platelet catecholamine levels and platelet aggregation.. J Hypertens Suppl, 7(6), S166-S167.
Ong A & Slater JD (1989) Intermittent absorption of warfarin caused by an unrecognized pharyngeal pouch.. Postgrad Med J, 65(767), 660-661.
Ong AC, Handler CE & Walker JM (1988) Hypersensitivity vasculitis complicating intravenous streptokinase therapy in acute myocardial infarction.. Int J Cardiol, 21(1), 71-73.
() Tubulointerstitial actions of endothelins in the kidney: roles in health and disease. Nephrology Dialysis Transplantation.
Patera F, Hautbergue GM, Wilson P, Evans PC, Ong ACM & Fragiadaki M () Ankhd1 enhances polycystic kidney disease development via promoting proliferation and fibrosis.

Chapters

Ong ACM & Sandford R (2015) Autosomal dominant polycystic kidney disease, Oxford Textbook of Clinical Nephrology (pp. 2625-2626). Oxford University Press
Ong ACM & Ellam T (2015) Autosomal dominant polycystic kidney disease, Oxford Textbook of Clinical Nephrology (pp. 2627-2633). Oxford University Press
Ong ACM & Maxwell AP (2014) Genetics and Genomics of Chronic Kidney Disease, Genomic Medicine (pp. 369-392). Oxford University Press
Streets A & Ong A (2014) TRPP2 in Polycystic Kidney Disease, Pathologies of Calcium Channels (pp. 491-522). Springer Berlin Heidelberg

Conference proceedings papers

Winterbottorn J, Simms R & Ong A (2020) REDUCED QUALITY OF LIFE IN ADPKD PATIENTS WITH CKD STAGE 1-3: THE CYSTIC I QUALITY OF LIFE STUDY. NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 35 (pp 98-98)
Magayr T, Streets A & Ong A (2020) SAT-442 Identification of exosome microRNAs as novel biomarkers for rapid disease progression in autosomal dominant polycystic kidney disease. Kidney International Reports, Vol. 5(3) (pp S185-S185). Abu Dhabi, United Arab Emirates, 26 March 2020 - 29 March 2020. View this article in WRRO
Magayr T, Streets A & Ong A (2019) GLOBAL MICRORNA PROFILING IN HUMAN URINARY EXOSOMES REVEALS NEW DISEASE BIOMARKERS AND CELLULAR PATHWAYS FOR AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD). NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 34
Huynh VT, Audrezet M-P, Domenger C, Bridoux F, Seret G, Longuet H, Halimi J-M, Senum S, Pajot A, Albaret J , Ponlot E et al (2019) CLINICAL PRESENTATION AND PROGNOSIS OF DNAJB11-ASSOCIATED NEPHROPATHY: AN INTERNATIONAL COLLABORATIVE STUDY. NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 34
Gimpel C, Avni EF, Breysem L, Burgmaier K, Caroli A, Cetiner M, Haffner D, Hartung EA, Franke D, König J , Liebau MC et al (2018) Imaging of kidney cysts and cystic kidney diseases in children. Consensus paper by an ad hoc committee. Ultraschall in der Medizin, 14 November 2018 - 16 November 2018. View this article in WRRO
Logeman C, Cho Y, Sautenet B, Rangan G, Gutman T, Craig J, Ong ACM, Chapman A, Ahn C, Coolican H , Kao JT-W et al (2018) PATIENT AND CAREGIVER BELIEFS, ATTITUDES AND PERSPECTIVES ON GENETIC SCREENING AND TESTING FOR AUTOSOMAL POLYCYSTIC KIDNEY DISEASE. NEPHROLOGY, Vol. 23 (pp 88-88)
Chong J, Durkie M, Dalton A & Ong A (2017) VERY EARLY-ONSET AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE DUE TO BI-ALLELIC MUTATIONS IN PKD1 AND PKD2. NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 32 (pp 94-94)
Simms RJ, Ryan D, Metherall P, Wright P, Gruel N, Tindale W & Ong ACM (2016) SO052DEVELOPMENT OF A RAPID SEMI-AUTOMATED TOOL TO MEASURE TOTAL KIDNEY VOLUME IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE. Nephrology Dialysis Transplantation, Vol. 31(suppl_1) (pp i22-i22) View this article in WRRO
Lannoy M, Abdela-ali F, Streets AJ & Ong ACM (2016) SELECTIVE PROSTAGLANDIN E2 RECEPTOR BLOCKADE FOR THE TREATMENT OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE. NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 31 (pp 22-23)
Rocío Cantero MD, Velázquez IF, Streets AJ, Ong ACM & Cantiello HF (2016) The Cyclic AMP Signaling Pathway and Direct PKA Phosphorylation Regulate Polycystin-2 (TRPP2) Channel Function. Biophysical Journal, Vol. 110(3) (pp 611a-612a)
Robinson P, McEwan P, Ong ACM, Ørskov B, Sandford R, Scolari F, Walz G, Bennet-Wilton H & O'Reilly K (2015) FP064ASSESSING THE LONG TERM OUTCOMES OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD) USING THE ADPKD OUTCOMES MODEL: A UK CASE STUDY. Nephrology Dialysis Transplantation, Vol. 30(suppl_3) (pp iii85-iii86)
Simms RJ, Thong KM, Dworschak GC & Ong ACM (2015) INCREASED PSYCHOSOCIAL BURDEN AND ADVERSE QUALITY OF LIFE IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE. NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 30
Streets AJ, Xu Y & Ong ACM (2015) POLYCYSTIN-1 TRAFFICKING IS REGULATED BY CAMP DEPENDENT PHOSPHORYLATION OF THE PLAT DOMAIN. NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 30
Robinson P, McEwan P, Hadimeri H, Ong ACM, Orskov B, Peces R, Sandford R, Scolari F, Walz G, Cooke C & O'Reilly K (2014) DEVELOPMENT OF A MODEL TO PREDICT DISEASE PROGRESSION IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD). NEPHROLOGY DIALYSIS TRANSPLANTATION, Vol. 29 (pp 75-75)
Holden BJ, van Eeden FJ, Ong ACM & Chico TJ (2011) PKD2 MUTANT ZEBRAFISH DISPLAY EXCESSIVE DEVELOPMENTAL ANGIOGENESIS. HEART, Vol. 97(20) (pp 12-12)
Streets AJ, Ibraghimov-Beskrovnaya O & Ong ACM (2003) Regulation of polycystin-1 mediated intercellular adhesion by phosphorylation in renal epithelia cells. JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, Vol. 14 (pp 575A-575A)
Robson L, Laycock SK, Brier T, Ong ACM & Haigh C (2003) A Ca2+-activated conductance in cultured mouse collecting duct cells.. JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, Vol. 14 (pp 80A-80A)
Zhao Y, Haylor JL & Ong ACM (2002) Upregulation of polycystin-2 expression following experimental ischaemic renal injury.. J AM SOC NEPHROL, Vol. 13 (pp 113A-113A)

Research group

Dr Andrew Streets
Dr Manoj Kumar Valluru
Dr Maryam Ahmadi
Ms Lisa Chang
Dr Jean Winterbottom
Dr Joshua Griffiths

PhD Students

Elliot Brooks (with Iwan Evans)
Jiehan Chong (with David Beech)

Teaching interests

Education and Training

I am Academic Programme Director for Nephrology and Deputy Director for Clinical Academic Training at the University of Sheffield. I previously served as a Board member of the EU FP7 TRANCYST initial training network and the ERA-EDTA Working Group on Inherited Kidney Diseases (WGIKD).

My wider educational interests concern the support and advancement of nephrology in the developing world. I am a member of the ISN Fellowship Committee and was previously Chair of the Renal Association International Committee (2009-2013).

I have hosted an ISN Sister Centre, several International Society of Nephrology (ISN) fellows and am an ISN Educational Ambassador.

Professional activities

National and International Committees

CYSTic Consortium – Chief Investigator.
ADPKD in Europe Consortium - Co-chair.
European ADPKD Forum – Faculty member.
ISN Fellowship committee.
UK Kidney Research Consortium (UKKRC) Clinical Study Group on Cystic Kidney Diseases - Chair (2009-2014), Deputy Chair (2014-2020)
Renal Association International Committee - Chair (2009-2013), Committee member.

Editorial Boards

Editor, Nature Scientific Reports.
Theme Editor, Nephrology Dialysis Transplantation.
Review Editor, Frontiers in Pharmacology.
Faculty of 1000 Medicine, Nephrology faculty.
Subject Editor, Nephrology.
Deputy Editor, Nephron Clinical Practice (2008-2012).
Associate Editor, BMC Nephrology (2008-2012).

Advisory Boards

ERA-EDTA Scientific Advisory Board.
Mayo Translational PKD Centre, Mayo Clinic, Rochester - External Faculty.
National Institute for Clinical Excellence (NICE) Technology Appraisal of Tolvaptan for ADPKD - Nominated Clinical Expert (2013-2015).
Renal Genomic England Clinical Interpretation Partnership (GeCIP) – Co-chair for Cystic Diseases.
Federation of European Associations of Patients Affected by Genetic Diseases (FEDERG) - Scientific Council.
UK Chinese Life Scientists Society – Fellow.

National and International Guidelines

Standardised Outcomes in Nephrology –PKD (SONG-PKD) – Steering committee.
PKD Outcomes Consortium (PKDOC) – Committee.
Renal Association Working Group for Tolvaptan in ADPKD – Chair.
ERA-EDTA Guidance for Tolvaptan in ADPKD – Committee.
KDIGO Controversies Conference on ADPKD – Invited participant and discussant (2014).