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Page 1
Fumarate Hydratase Deletion in Pancreatic β Cells Leads to Progressive Diabetes.
Adam J, Ramracheya R, Chibalina MV, Ternette N, Hamilton A, Tarasov AI, Zhang Q, Rebelato E, Rorsman NJG, Martín-Del-Río R, Lewis A, Özkan G, Do HW, Spégel P, Saitoh K, Kato K, Igarashi K, Kessler BM, Pugh CW, Tamarit-Rodriguez J, Mulder H, Clark A, Frizzell N, Soga T, Ashcroft FM, Silver A, Pollard PJ, Rorsman P. Adam J, et al. Among authors: pugh cw. Cell Rep. 2017 Sep 26;20(13):3135-3148. doi: 10.1016/j.celrep.2017.08.093. Cell Rep. 2017. PMID: 28954230 Free PMC article.
Dysregulation of hypoxia pathways in fumarate hydratase-deficient cells is independent of defective mitochondrial metabolism.
O'Flaherty L, Adam J, Heather LC, Zhdanov AV, Chung YL, Miranda MX, Croft J, Olpin S, Clarke K, Pugh CW, Griffiths J, Papkovsky D, Ashrafian H, Ratcliffe PJ, Pollard PJ. O'Flaherty L, et al. Among authors: pugh cw. Hum Mol Genet. 2010 Oct 1;19(19):3844-51. doi: 10.1093/hmg/ddq305. Epub 2010 Jul 21. Hum Mol Genet. 2010. PMID: 20660115 Free PMC article.
Expression profiling in progressive stages of fumarate-hydratase deficiency: the contribution of metabolic changes to tumorigenesis.
Ashrafian H, O'Flaherty L, Adam J, Steeples V, Chung YL, East P, Vanharanta S, Lehtonen H, Nye E, Hatipoglu E, Miranda M, Howarth K, Shukla D, Troy H, Griffiths J, Spencer-Dene B, Yusuf M, Volpi E, Maxwell PH, Stamp G, Poulsom R, Pugh CW, Costa B, Bardella C, Di Renzo MF, Kotlikoff MI, Launonen V, Aaltonen L, El-Bahrawy M, Tomlinson I, Pollard PJ. Ashrafian H, et al. Among authors: pugh cw. Cancer Res. 2010 Nov 15;70(22):9153-65. doi: 10.1158/0008-5472.CAN-10-1949. Epub 2010 Oct 26. Cancer Res. 2010. PMID: 20978192
Aberrant succination of proteins in fumarate hydratase-deficient mice and HLRCC patients is a robust biomarker of mutation status.
Bardella C, El-Bahrawy M, Frizzell N, Adam J, Ternette N, Hatipoglu E, Howarth K, O'Flaherty L, Roberts I, Turner G, Taylor J, Giaslakiotis K, Macaulay VM, Harris AL, Chandra A, Lehtonen HJ, Launonen V, Aaltonen LA, Pugh CW, Mihai R, Trudgian D, Kessler B, Baynes JW, Ratcliffe PJ, Tomlinson IP, Pollard PJ. Bardella C, et al. Among authors: pugh cw. J Pathol. 2011 Sep;225(1):4-11. doi: 10.1002/path.2932. Epub 2011 Jun 1. J Pathol. 2011. PMID: 21630274
Renal cyst formation in Fh1-deficient mice is independent of the Hif/Phd pathway: roles for fumarate in KEAP1 succination and Nrf2 signaling.
Adam J, Hatipoglu E, O'Flaherty L, Ternette N, Sahgal N, Lockstone H, Baban D, Nye E, Stamp GW, Wolhuter K, Stevens M, Fischer R, Carmeliet P, Maxwell PH, Pugh CW, Frizzell N, Soga T, Kessler BM, El-Bahrawy M, Ratcliffe PJ, Pollard PJ. Adam J, et al. Among authors: pugh cw. Cancer Cell. 2011 Oct 18;20(4):524-37. doi: 10.1016/j.ccr.2011.09.006. Cancer Cell. 2011. PMID: 22014577 Free PMC article.
A role for cytosolic fumarate hydratase in urea cycle metabolism and renal neoplasia.
Adam J, Yang M, Bauerschmidt C, Kitagawa M, O'Flaherty L, Maheswaran P, Özkan G, Sahgal N, Baban D, Kato K, Saito K, Iino K, Igarashi K, Stratford M, Pugh C, Tennant DA, Ludwig C, Davies B, Ratcliffe PJ, El-Bahrawy M, Ashrafian H, Soga T, Pollard PJ. Adam J, et al. Cell Rep. 2013 May 30;3(5):1440-8. doi: 10.1016/j.celrep.2013.04.006. Epub 2013 May 2. Cell Rep. 2013. PMID: 23643539 Free PMC article.
158 results