Items where authors include "Ong, A.C.M."

Article

Cipriani, V. orcid.org/0000-0002-0839-9955, Vestito, L. orcid.org/0000-0003-0008-936X, Magavern, E.F. orcid.org/0000-0003-0699-6411 et al. (58 more authors) (2025) Rare disease gene association discovery in the 100,000 Genomes Project. Nature. ISSN 0028-0836

Gittus, M., Haley, H., Harris, T. et al. (10 more authors) (2025) Commentary: Tolvaptan for Autosomal Dominant Polycystic Kidney Disease (ADPKD) - an update. BMC Nephrology, 26 (1). 79. ISSN 1471-2369

Devuyst, O. orcid.org/0000-0003-3744-4767, Ahn, C., Barten, T.R.M. et al. (24 more authors) (2025) KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD). Kidney International, 107 (2). S1-S239. ISSN 0085-2538

Torres, V.E., Ahn, C., Barten, T.R.M. et al. (27 more authors) (2025) KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary. Kidney International, 107 (2). pp. 234-254. ISSN 0085-2538

Zagorec, N., Calamel, A., Delaporte, M. et al. (24 more authors) (2024) Clinical spectrum and prognosis of atypical autosomal dominant polycystic kidney disease caused by monoallelic pathogenic variants of IFT140. American Journal of Kidney Diseases. ISSN 0272-6386

Bais, T., Meijer, E. orcid.org/0000-0001-7875-1793, Kramers, B.J. et al. (15 more authors) (2024) HYDROchlorothiazide versus placebo to PROTECT polycystic kidney disease patients and improve their quality of life: study protocol and rationale for the HYDRO-PROTECT randomized controlled trial. Trials, 25 (1). 120. ISSN 1745-6215

Francis, A., Harhay, M.N., Ong, A.C.M. orcid.org/0000-0002-7211-5400 et al. (16 more authors) (2024) Chronic kidney disease and the global public health agenda: an international consensus. Nature Reviews Nephrology, 20 (7). pp. 473-485. ISSN 1759-5061

Wong, K., Pitcher, D., Braddon, F. et al. (299 more authors) (2024) Description and Cross-Sectional Analyses of 25,880 Adults and Children in the UK National Registry of Rare Kidney Diseases Cohort. Kidney International Reports, 9 (7). pp. 2067-2083. ISSN 2468-0249

Wong, K., Pitcher, D., Braddon, F. et al. (299 more authors) (2024) Description and cross-sectional analyses of 25,880 adults and children in the UK National Registry of rare kidney diseases cohort. Kidney International Reports, 9 (7). pp. 2067-2083. ISSN 2468-0249

Chen, E.W.C., Chong, J., Valluru, M.K. orcid.org/0000-0001-9156-866X et al. (4 more authors) (2024) Combining genotype with height-adjusted kidney length predicts rapid progression of ADPKD. Nephrology Dialysis Transplantation, 39 (6). pp. 956-966. ISSN 0931-0509

Fung, W.W.-S. orcid.org/0000-0001-7676-7628, Szeto, C.-C. orcid.org/0000-0002-8898-8505, Chow, K.-M. orcid.org/0000-0001-5310-5197 et al. (8 more authors) (2024) Clinical characteristics and kidney outcomes in Chinese patients with autosomal dominant polycystic kidney disease. Kidney360, 5 (5). pp. 715-723. ISSN 2641-7650

Wong, K., Pitcher, D., Braddon, F. et al. (294 more authors) (2024) Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort. The Lancet, 403 (10433). pp. 1279-1289. ISSN 0140-6736

Wong, K., Pitcher, D., Braddon, F. et al. (294 more authors) (2024) Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort. The Lancet, 403 (10433). pp. 1279-1289. ISSN 0140-6736

Ali, H. orcid.org/0000-0001-6623-3374, Malik, M.Z., Abu‐Farha, M. et al. (12 more authors) (2024) Global analysis of urinary extracellular vesicle small RNAs in autosomal dominant polycystic kidney disease. The Journal of Gene Medicine, 26 (2). e3674. ISSN 1099-498X

Taylor, J., Thomas, R., Metherall, P. et al. (15 more authors) (2024) An artificial intelligence generated automated algorithm to measure total kidney volume in ADPKD. Kidney International Reports, 9 (2). pp. 249-256. ISSN 2468-0249

Brooks, E.C., Zeidler, M.P., Ong, A.C.M. orcid.org/0000-0002-7211-5400 et al. (1 more author) (2024) Macrophage subpopulation identity in Drosophila is modulated by apoptotic cell clearance and related signalling pathways. Frontiers in Immunology, 14. 1310117. ISSN 1664-3224

Gittus, M. orcid.org/0000-0002-8406-2027, Moore, J. and Ong, A.C.M. (2024) Liver transplant recipients with polycystic liver disease have longer waiting times but better long-term clinical outcomes than those with liver disease due to other causes: a retrospective cross-sectional study. PLOS ONE, 19 (1). e0294717. ISSN 1932-6203

Gittus, M. orcid.org/0000-0002-8406-2027, Chong, J., Sutton, A. et al. (2 more authors) (2023) Barriers and facilitators to the implementation of guidelines in rare diseases: a systematic review. Orphanet Journal of Rare Diseases, 18 (1). 140. ISSN 1750-1172

Harskamp, L.R., Perez-Gomez, M.V. orcid.org/0000-0003-4558-5236, Heida, J.E. et al. (16 more authors) (2023) The association of urinary epidermal growth factors with ADPKD disease severity and progression. Nephrology Dialysis Transplantation, 38 (10). pp. 2266-2275. ISSN 0931-0509

Wang, Q., Zou, B., Wei, X. et al. (10 more authors) (2023) Identification of renal cyst cells of type I Nephronophthisis by single-nucleus RNA sequencing. Frontiers in Cell and Developmental Biology, 11. 1192935. ISSN 2296-634X

Durkie, M., Watson, C.M. orcid.org/0000-0003-2371-1844, Winship, P. et al. (10 more authors) (2023) The common PKD1 p.(Ile3167Phe) variant is hypomorphic and associated with very early onset, biallelic polycystic kidney disease. Human Mutation, 2023. 5597005. ISSN 1059-7794

Cadnapaphornchai, M.A. and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2023) Hypertension in young adults with autosomal dominant polycystic kidney disease: a case for early screening? Clinical Kidney Journal, 16 (6). pp. 901-904. ISSN 2048-8505

Perrone, R.D. orcid.org/0000-0002-4963-1132, Oberdhan, D. orcid.org/0000-0003-1034-9049, Ouyang, J. et al. (9 more authors) (2023) OVERTURE: A worldwide, prospective, observational study of disease characteristics in patients with ADPKD. Kidney International Reports, 8 (5). pp. 989-1001. ISSN 2468-0249

Gittus, M., Harris, T. orcid.org/0000-0003-3659-7438 and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2023) Patient perspectives on ADPKD. Advances in Kidney Disease and Health, 30 (3). pp. 294-302. ISSN 2949-8139

Gansevoort, R.T., Hariri, A., Minini, P. et al. (11 more authors) (2023) Venglustat, a novel glucosylceramide synthase inhibitor, in patients at risk of rapidly progressing ADPKD: primary results of a double-blind, placebo-controlled, phase 2/3 randomized clinical trial. American Journal of Kidney Diseases, 81 (5). 517-527.e1. ISSN 0272-6386

Valluru, M.K. orcid.org/0000-0001-9156-866X, Chung, N.K.X., Gilchrist, M. et al. (6 more authors) (2023) A founder UMOD variant is a common cause of hereditary nephropathy in the British population. Journal of Medical Genetics, 60 (4). pp. 397-405. ISSN 0022-2593

Lambert, K., Gardos, R., Coolican, H. et al. (4 more authors) (2023) Diet and polycystic kidney disease: nutrients, foods, dietary patterns, and implications for practice. Seminars in Nephrology, 43 (2). 151405. ISSN 0270-9295

Chong, J., Harris, T. and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2023) Regional variation in tolvaptan prescribing across England: national data and retrospective evaluation from an expert centre. Clinical Kidney Journal, 16 (1). pp. 61-68. ISSN 2048-8505

Winterbottom, J., Simms, R.J., Caroli, A. et al. (10 more authors) (2022) Flank pain has a significant adverse impact on quality of life in ADPKD: the CYSTic-QoL study. Clinical Kidney Journal, 15 (11). pp. 2063-2071. ISSN 2048-8505

Perrone, R.D., Hariri, A., Minini, P. et al. (10 more authors) (2022) The STAGED-PKD 2-stage adaptive study with a patient enrichment strategy and treatment effect modeling for improved study design efficiency in patients with ADPKD. Kidney Medicine, 4 (10). 100538. ISSN 2590-0595

Ong, A.C.M. orcid.org/0000-0002-7211-5400 and Torra, R. (2022) Can ketogenic dietary interventions slow disease progression in ADPKD : what we know and what we don't. Clinical Kidney Journal, 15 (6). sfac103. pp. 1034-1036. ISSN 2048-8505

Chung, N.K.X., Metherall, P., McCormick, J.A. et al. (2 more authors) (2022) Individualized everolimus treatment for tuberous sclerosis-related angiomyolipoma promotes treatment adherence and response. Clinical Kidney Journal, 15 (6). pp. 1160-1168. ISSN 2048-8505

Müller, R.-U., Lianne Messchendorp, A., Birn, H. et al. (20 more authors) (2022) An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease International. Kidneys, 11 (1). pp. 37-39. ISSN 2307-1257

Chang, M.-Y., Tsai, T.-I., Chou, L.-F. et al. (6 more authors) (2022) Metformin induces lactate accumulation and accelerates renal cyst progression in Pkd1-deficient mice. Human Molecular Genetics, 31 (10). pp. 1560-1573. ISSN 0964-6906

Müller, R.-U., Messchendorp, A.L., Birn, H. et al. (20 more authors) (2022) An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease International. Nephrology Dialysis Transplantation, 37 (5). pp. 825-839. ISSN 0931-0509

Li, D., Hu, M., Chen, H. et al. (9 more authors) (2022) An Nphp1 knockout mouse model targeting exon 2–20 demonstrates characteristic phenotypes of human Nephronophthisis. Human Molecular Genetics, 31 (2). pp. 232-243. ISSN 0964-6906

Senum, S.R., Li, Y.S.M., Benson, K.A. et al. (25 more authors) (2022) Monoallelic IFT140 pathogenic variants are an important cause of the autosomal dominant polycystic kidney-spectrum phenotype. The American Journal of Human Genetics, 109 (1). pp. 136-156. ISSN 0002-9297

Ong, A.C.M. orcid.org/0000-0002-7211-5400 and Gansevoort, R.T. (2021) TAMEing ADPKD with metformin : safe and effective? Kidney International, 100 (3). pp. 513-515. ISSN 0085-2538

Mao, Z., Valluru, M.K. orcid.org/0000-0001-9156-866X and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2021) Drug repurposing in autosomal dominant polycystic kidney disease: back to the future with pioglitazone. Clinical Kidney Journal, 14 (7). pp. 1715-1718. ISSN 2048-8505

Durkie, M., Chong, J., Valluru, M.K. et al. (2 more authors) (2021) Biallelic inheritance of hypomorphic PKD1 variants is highly prevalent in very early onset polycystic kidney disease. Genetics in Medicine, 23 (4). pp. 689-697. ISSN 1098-3600

Cho, Y., Tong, A., Craig, J.C. et al. (28 more authors) (2021) 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, 77 (2). pp. 255-263. ISSN 0272-6386

Zhou, S., Xu, J., Xue, C. et al. (3 more authors) (2021) Coronavirus-associated kidney outcomes in COVID-19, SARS, and MERS: a meta-analysis and systematic review. Renal Failure, 43 (1). pp. 1-15. ISSN 0886-022X

Fragiadaki, M. orcid.org/0000-0002-1587-5577, Macleod, F.M. and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2020) The controversial role of fibrosis in Autosomal Dominant Polycystic Kidney Disease. International Journal of Molecular Sciences, 21 (23). 8936. ISSN 1661-6596

Cho, Y., Rangan, G., Logeman, C. et al. (25 more authors) (2020) Core outcome domains for trials in autosomal dominant polycystic kidney disease: An international Delphi survey. American Journal of Kidney Diseases, 76 (3). pp. 361-373. ISSN 0272-6386

Streets, A.J. orcid.org/0000-0002-4328-044X, Prosseda, P.P. and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2020) Polycystin-1 regulates ARHGAP35-dependent centrosomal RhoA activation and ROCK signaling. JCI Insight, 5 (16). e135385.

Magayr, T.A., Song, X., Streets, A.J. orcid.org/0000-0002-4328-044X et al. (10 more authors) (2020) Global microRNA profiling in human urinary exosomes reveals novel disease biomarkers and cellular pathways for Autosomal Dominant Polycystic Kidney Disease. Kidney International, 98 (2). pp. 420-435. ISSN 0085-2538

Lannoy, M., Valluru, M.K., Chang, L. et al. (4 more authors) (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, 98 (2). pp. 404-419. ISSN 0085-2538

Torres, V.E. and Ong, A.C.M. (2020) Cellular signaling in PKD: foreword. Cellular Signalling, 71. 109625. ISSN 0898-6568

Hopp, K. orcid.org/0000-0002-2420-2358, Cornec-Le Gall, E., Senum, S.R. orcid.org/0000-0002-5489-9117 et al. (17 more authors) (2020) Detection and characterization of mosaicism in autosomal dominant polycystic kidney disease. Kidney International, 97 (2). pp. 370-382. ISSN 0085-2538

Tam, F.W.K. and Ong, A.C.M. orcid.org/0000-0002-7211-5400 (2020) Renal monocyte chemoattractant protein-1: an emerging universal biomarker and therapeutic target for kidney diseases? Nephrology Dialysis Transplantation, 35 (2). pp. 198-203. ISSN 0931-0509

Metzner, A., Griffiths, J.D., Streets, A.J. et al. (4 more authors) (2020) A high throughput zebrafish chemical screen reveals ALK5 and non-canonical androgen signalling as modulators of the pkd2−/− phenotype. Scientific Reports, 10 (1). 72.

Annear, N.M.P., Appleton, R.E., Bassi, Z. et al. (21 more authors) (2019) Tuberous sclerosis complex (TSC) : expert recommendations for provision of coordinated care. Frontiers in Neurology, 10. 1116.

Simms, R.J., Doshi, T., Metherall, P. et al. (7 more authors) (2019) A rapid high-performance semi-automated tool to measure total kidney volume from MRI in autosomal dominant polycystic kidney disease. European Radiology, 29 (8). pp. 4188-4197. ISSN 0938-7994

Omar, F., Findlay, J.E., Carfray, G. et al. (19 more authors) (2019) Small-molecule allosteric activators of PDE4 long form cyclic AMP phosphodiesterases. Proceedings of the National Academy of Sciences, 116 (27). pp. 13320-13329. ISSN 0027-8424

Gimpel, C. orcid.org/0000-0003-1296-9081, Avni, E.F., Breysem, L. et al. (15 more authors) (2019) Imaging of kidney cysts and cystic kidney diseases in children: an international working group consensus statement. Radiology, 290 (3). pp. 769-782. ISSN 0033-8419

Selby, N., Blankestijn, P., Boor, P. et al. (31 more authors) (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 (Suppl 2). ii4-ii14. ISSN 0931-0509

Neagu, M., Coca, D. orcid.org/0000-0003-2878-2422 and Ong, A.C.M. (2018) Linear and Nonlinear Estimated GFR Slopes in ADPKD Patients Reaching ESRD. American Journal of Kidney Diseases, 71 (6). pp. 912-913. ISSN 0272-6386

Harris, T., Sandford, R., de Coninck, B. et al. (17 more authors) (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). pp. 563-573. ISSN 0931-0509

McEwan, P., Wilton, H.B., Ong, A.C.M. et al. (7 more authors) (2018) A model to predict disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD): the ADPKD Outcomes Model. BMC Nephrology, 19. 37.

Cho, Y. orcid.org/0000-0002-3502-9837, Sautenet, B., Rangan, G. et al. (21 more authors) (2017) Standardised outcomes in nephrology-polycystic kidney disease (SONG-PKD): study protocol for establishing a core outcome set in polycystic kidney disease. Trials, 18. 560. ISSN 1745-6215

Streets, A.J., Magayr, T.A., Huang, L. et al. (6 more authors) (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. ISSN 1931-857X

Fragiadaki, M., Lannoy, M., Themanns, M. et al. (5 more authors) (2017) STAT5 drives abnormal proliferation in autosomal dominant polycystic kidney disease. Kidney International. ISSN 0085-2538

Mao, Z., Chong, J. orcid.org/0000-0002-5846-7397 and Ong, A.C.M. (2016) Autosomal dominant polycystic kidney disease: recent advances in clinical management. F1000Research, 5. p. 2029. ISSN 2046-1402

Gansevoort, R.T., Arici, M., Benzing, T. et al. (18 more authors) (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). pp. 337-348. ISSN 0931-0509

Ong, A.C.M. and Harris, P.C. (2015) A polycystin-centric view of cyst formation and disease: the polycystins revisited. Kidney International, 88 (4). pp. 699-710. ISSN 0085-2538

Liu, Y., Kaech, A., Ziegler, U. et al. (4 more authors) (2013) The Role of Phospholipase D in Modulating the MTOR Signaling Pathway in Polycystic Kidney Disease. PLoS ONE, 8 (8). ARTN e73173. ISSN 1932-6203

Loftus, H. and Ong, A.C.M. (2012) Cystic kidney diseases: many ways to form a cyst. Pediatric Nephrology, 28 (1). 33 - 49. ISSN 0931-041X

Giamarchi, A., Feng, S., Rodat-Despoix, L. et al. (12 more authors) (2010) A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes. The EMBO Journal, 29 (7). pp. 1176-1191. ISSN 0261-4189

Yu, Y., Ulbrich, M., Li, M.H. et al. (6 more authors) (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). pp. 11558-11563. ISSN 0027-8424

Mao, Z. and Ong, A.C.M. (2009) Peroxisome proliferator-activated receptor gamma agonists in kidney disease - future promise, present fears. Nephron Clinical Practice, 112 (4). pp. 230-241. ISSN 1660-2110

Streets, A.J., Wagner, B.E., Harris, P.C. et al. (2 more authors) (2009) Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells. Journal of Cell Science, 122 (9). pp. 1410-1417. ISSN 0021-9533

Prasad, S., McDaid, J.P., Tam, F.W.K. et al. (2 more authors) (2009) Pkd2 dosage influences cellular repair responses following ischemia-reperfusion injury. American Journal of Pathology, 175 (4). pp. 1493-1503. ISSN 0002-9440

Ong, A.C.M. (2009) Screening for intracranial aneurysms in ADPKD. British Medical Journal, 339. Art no.b3763. ISSN 0959-8138

Feng, S., Okenka, G.M., Bai, C.X. et al. (6 more authors) (2008) Identification and functional characterization of an N-terminal oligomerization domain for polycystin-2*. Journal of Biological Chemistry, 283 (42). pp. 28471-28479. ISSN 0021-9258

Parker, E., Newby, L.J., Sharpe, C.C. et al. (5 more authors) (2007) Insulin-like growth factor-1 induces hyperproliferation of PKD1 cystic cells via a Ras/Raf dependent signalling pathway. Kidney International, 72 (2). pp. 157-165. ISSN 0085-2538

Streets, A.J., Moon, D.J., Kane, M.E. et al. (2 more authors) (2006) Identification of an N-terminal glycogen synthase kinase 3 phosphorylation site which regulates the functional localisation of polycystin-2 in vivo and in vitro. Human Molecular Genetics, 15 (9). pp. 1465-1473. ISSN 0964-6906

Le, N.H., van der Bent, P., Huls, G. et al. (8 more authors) (2004) Aberrant Polycystin-1 Expression Results in Modification of Activator Protein-1 Activity, whereas Wnt Signaling Remains Unaffected. Journal of Biological Chemistry, 279 (26). pp. 27472-27481. ISSN 1083-351X

Newby, L.J., Streets, A.J., Zhao, Y. et al. (3 more authors) (2002) Identification, Characterization, and Localization of a Novel Kidney Polycystin-1-Polycystin-2 Complex. Journal of Biological Chemistry, 277 (73). 20763 - 20773. ISSN 1083-351X

Proceedings Paper

Gimpel, C., Avni, E.F., Breysem, L. et al. (14 more authors) (2018) Imaging of kidney cysts and cystic kidney diseases in children. Consensus paper by an ad hoc committee. In: Ultraschall in der Medizin. Interdisziplinärer Kongress Ultraschall 2018, 14-16 Nov 2018, Basel, Switzerland. Georg Thieme Verlag KG , pp. 34-35.

Simms, R.J., Ryan, D., Metherall, P. et al. (4 more authors) (2016) Development of a rapid semi-automated tool to measure total kidney volume in autosomal dominant polycystic kidney disease. In: Nephrology Dialysis Transplantation. 53rd ERA-EDTA Congress, May 21st-24th, 2016, Vienna, Austria. , p. 22.

Lannoy, M., Abdela-ali, F., Streets, A.J. orcid.org/0000-0002-4328-044X et al. (1 more author) (2016) SO053: Selective elective prostaglandin E2 receptor blockade for the treatment of autosomal dominant polycystic kidney disease. In: Nephrology Dialysis Transplantation. 53rd ERA-EDTA Congress, 21-24 May 2016, Vienna, Austria. Oxford University Press (OUP) , i22-i23.