تفاوت میان نسخه‌های «پروتئین متصل‌شونده به E3»

جز
ربات:مرتب‌سازی عنوان‌ها+تمیز+
جز (انتقال خودکار میان‌ویکی به ویکی‌داده)
جز (ربات:مرتب‌سازی عنوان‌ها+تمیز+)
{{Infobox gene}}
'''پروتئین متصل‌شونده به E3''' ({{lang-en|E3 binding protein}}) که با نام '''پروتئین پیرووات دهیدروژناز، جزء ساختمانی X، میتوکندریایی''' هم شناخته می‌شود یک [[پروتئین]] است که در انسان توسط ژن «''PDHX''» کُدگذاری می‌شود.<ref name="entrez">{{cite web | title = Entrez Gene: pyruvate dehydrogenase complex| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=8050| accessdate = }}</ref><ref name="pmid9399911">{{cite journal | vauthors = Aral B, Benelli C, Ait-Ghezala G, Amessou M, Fouque F, Maunoury C, Créau N, Kamoun P, Marsac C | title = Mutations in PDX1, the human lipoyl-containing component X of the pyruvate dehydrogenase-complex gene on chromosome 11p1, in congenital lactic acidosis | journal = American Journal of Human Genetics | volume = 61 | issue = 6 | pages = 1318–26 | date = Dec 1997 | pmid = 9399911 | pmc = 1716072 | doi = 10.1086/301653 }}</ref><ref name="pmid9467010">{{cite journal | vauthors = Ling M, McEachern G, Seyda A, MacKay N, Scherer SW, Bratinova S, Beatty B, Giovannucci-Uzielli ML, Robinson BH | title = Detection of a homozygous four base pair deletion in the protein X gene in a case of pyruvate dehydrogenase complex deficiency | journal = Human Molecular Genetics | volume = 7 | issue = 3 | pages = 501–5 | date = Mar 1998 | pmid = 9467010 | doi = 10.1093/hmg/7.3.501 }}</ref><ref name="pmid10568747">{{cite journal | vauthors = Gawin B, Niederführ A, Schumacher N, Hummerich H, Little PF, Gessler M | title = A 7.5 Mb sequence-ready PAC contig and gene expression map of human chromosome 11p13-p14.1 | journal = Genome Research | volume = 9 | issue = 11 | pages = 1074–86 | date = Nov 1999 | pmid = 10568747 | pmc = 310838 | doi = 10.1101/gr.9.11.1074 }}</ref> این پروتئین بخشی از کمپلکس پیرووات دهیدروژناز است که در [[یوکاریوت]]‌ها یافت می‌شود.
 
== اهمیت بالینی ==
جهش در ژن «''PDHX''» سبب بروز یکی از انواع [[کمبود پیرووات دهیدروژناز]] می‌گردد. این پروتئین همچنین یک آنتی‌ژن ضعیف برای «آنتی‌بادی‌های ضد میتوکندری» (AMA) محسوب می‌شود که در ۹۵٪ بیماران مبتلا به «[[سیروز صفراوی]] اولیه» (PBC) یافت می‌شود و در نهایت منجر به [[سیروز کبدی|سیروز]] و نارسایی کبدی می‌گردد.<ref name="entrez"/><ref>{{cite journal | vauthors = McHugh A, Robe AJ, Palmer JM, Jones DE | title = PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis | journal = Liver International | volume = 26 | issue = 4 | pages = 406–13 | date = May 2006 | pmid = 16629643 | doi = 10.1111/j.1478-3231.2006.01253.x }}</ref>
 
== منابع ==
{{چپ‌چین}}
{{refbegin|33em}}
* {{cite journal | vauthors = Qiu Y, Guo M, Huang S, Stein R | title = Insulin gene transcription is mediated by interactions between the p300 coactivator and PDX-1, BETA2, and E47 | journal = Molecular and Cellular Biology | volume = 22 | issue = 2 | pages = 412–20 | date = Jan 2002 | pmid = 11756538 | pmc = 139753 | doi = 10.1128/mcb.22.2.412-420.2002 }}
* {{cite journal | vauthors = Brown RM, Head RA, Morris AA, Raiman JA, Walter JH, Whitehouse WP, Brown GK | title = Pyruvate dehydrogenase E3 binding protein (protein X) deficiency | journal = Developmental Medicine and Child Neurology | volume = 48 | issue = 9 | pages = 756–60 | date = Sep 2006 | pmid = 16904023 | doi = 10.1017/S0012162206001617 }}
* {{cite journal | vauthors = Brown RM, Head RA, Brown GK | title = Pyruvate dehydrogenase E3 binding protein deficiency | journal = Human Genetics | volume = 110 | issue = 2 | pages = 187–91 | date = Feb 2002 | pmid = 11935326 | doi = 10.1007/s00439-001-0665-3 }}
* {{cite journal | vauthors = Ciszak EM, Makal A, Hong YS, Vettaikkorumakankauv AK, Korotchkina LG, Patel MS | title = How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex | journal = The Journal of Biological Chemistry | volume = 281 | issue = 1 | pages = 648–55 | date = Jan 2006 | pmid = 16263718 | doi = 10.1074/jbc.M507850200 }}
* {{cite journal | vauthors = Kimura K, Wakamatsu A, Suzuki Y, Ota T, Nishikawa T, Yamashita R, Yamamoto J, Sekine M, Tsuritani K, Wakaguri H, Ishii S, Sugiyama T, Saito K, Isono Y, Irie R, Kushida N, Yoneyama T, Otsuka R, Kanda K, Yokoi T, Kondo H, Wagatsuma M, Murakawa K, Ishida S, Ishibashi T, Takahashi-Fujii A, Tanase T, Nagai K, Kikuchi H, Nakai K, Isogai T, Sugano S | title = Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes | journal = Genome Research | volume = 16 | issue = 1 | pages = 55–65 | date = Jan 2006 | pmid = 16344560 | pmc = 1356129 | doi = 10.1101/gr.4039406 }}
* {{cite journal | vauthors = Brautigam CA, Wynn RM, Chuang JL, Machius M, Tomchick DR, Chuang DT | title = Structural insight into interactions between dihydrolipoamide dehydrogenase (E3) and E3 binding protein of human pyruvate dehydrogenase complex | journal = Structure | volume = 14 | issue = 3 | pages = 611–21 | date = Mar 2006 | pmid = 16442803 | pmc = 2879633 | doi = 10.1016/j.str.2006.01.001 }}
* {{cite journal | vauthors = Sugden MC, Holness MJ | title = Recent advances in mechanisms regulating glucose oxidation at the level of the pyruvate dehydrogenase complex by PDKs | journal = American Journal of Physiology. Endocrinology and Metabolism | volume = 284 | issue = 5 | pages = E855-62 | date = May 2003 | pmid = 12676647 | doi = 10.1152/ajpendo.00526.2002 }}
* {{cite journal | vauthors = Schiff M, Miné M, Brivet M, Marsac C, Elmaleh-Bergés M, Evrard P, Ogier de Baulny H | title = Leigh's disease due to a new mutation in the PDHX gene | journal = Annals of Neurology | volume = 59 | issue = 4 | pages = 709–14 | date = Apr 2006 | pmid = 16566017 | doi = 10.1002/ana.20818 }}
* {{cite journal | vauthors = Miné M, Brivet M, Schiff M, de Baulny HO, Chuzhanova N, Marsac C | title = A novel gross deletion caused by non-homologous recombination of the PDHX gene in a patient with pyruvate dehydrogenase deficiency | journal = Molecular Genetics and Metabolism | volume = 89 | issue = 1-2 | pages = 106–10 | year = 2006 | pmid = 16843025 | doi = 10.1016/j.ymgme.2006.06.002 }}
* {{cite journal | vauthors = Murray J, Gilkerson R, Capaldi RA | title = Quantitative proteomics: the copy number of pyruvate dehydrogenase is more than 10(2)-fold lower than that of complex III in human mitochondria | journal = FEBS Letters | volume = 529 | issue = 2-3 | pages = 173–8 | date = Oct 2002 | pmid = 12372595 | doi = 10.1016/S0014-5793(02)03278-7 }}
* {{cite journal | vauthors = Hiromasa Y, Roche TE | title = Facilitated interaction between the pyruvate dehydrogenase kinase isoform 2 and the dihydrolipoyl acetyltransferase | journal = The Journal of Biological Chemistry | volume = 278 | issue = 36 | pages = 33681–93 | date = Sep 2003 | pmid = 12816949 | doi = 10.1074/jbc.M212733200 }}
* {{cite journal | vauthors = Hiromasa Y, Fujisawa T, Aso Y, Roche TE | title = Organization of the cores of the mammalian pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and their capacities to bind the E1 and E3 components | journal = The Journal of Biological Chemistry | volume = 279 | issue = 8 | pages = 6921–33 | date = Feb 2004 | pmid = 14638692 | doi = 10.1074/jbc.M308172200 }}
* {{cite journal | vauthors = Boulatnikov I, Popov KM | title = Formation of functional heterodimers by isozymes 1 and 2 of pyruvate dehydrogenase kinase | journal = Biochimica et Biophysica Acta | volume = 1645 | issue = 2 | pages = 183–92 | date = Feb 2003 | pmid = 12573248 | doi = 10.1016/S1570-9639(02)00542-3 }}
* {{cite journal | vauthors = McHugh A, Robe AJ, Palmer JM, Jones DE | title = PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis | journal = Liver International | volume = 26 | issue = 4 | pages = 406–13 | date = May 2006 | pmid = 16629643 | doi = 10.1111/j.1478-3231.2006.01253.x }}
* {{cite journal | vauthors = Bottone MG, Soldani C, Fraschini A, Alpini C, Croce AC, Bottiroli G, Pellicciari C | title = Enzyme-assisted photosensitization with rose Bengal acetate induces structural and functional alteration of mitochondria in HeLa cells | journal = Histochemistry and Cell Biology | volume = 127 | issue = 3 | pages = 263–71 | date = Mar 2007 | pmid = 17024456 | doi = 10.1007/s00418-006-0235-9 }}
{{refend}}
{{پایان چپ‌چین}}
== پیوند به بیرون ==
* {{MeshName|PDHX+protein,+human}}
{{PDB Gallery|geneid=8050}}
 
{{زیست‌شناسی مولکولی-خرد}}
{{PDB Gallery|geneid=8050}}
 
[[رده:پروتئین‌های میتوکندریایی]]
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