{{위키데이터 속성 추적}}
{{Infobox_gene}}
'''가지사슬 케토산 탈수소효소 키네이스'''({{llang|en|branched chain ketoacid dehydrogenase kinase}}, '''BCKDK''')는 [[16번 염색체]]에 있는 BCKDK [[유전자]]에 의해 암호화되는 [[효소]]이다. 이 효소는 미토콘드리아 [[단백질 키네이스]] 패밀리의 일부이며 [[발린]], [[류신]], [[아이소류신]]의 [[이화 경로]]의 조절자이다.<ref name="entrez">{{웹 인용| title = Entrez Gene: BCKDK branched chain ketoacid dehydrogenase kinase| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=10295}}</ref> BCKDK는 [[미토콘드리아 기질]]에서 발견되며 그 유병률은 [[세포]] 유형에 따라 다르다. [[간세포]]는 BCKDK의 농도가 가장 낮은 경향이 있는 반면, [[골격근 세포]]는 가장 높은 농도를 가지고 있다.<ref name="pmid1377677">{{저널 인용| vauthors = Popov KM, Zhao Y, Shimomura Y, Kuntz MJ, Harris RA | title = Branched-chain alpha-ketoacid dehydrogenase kinase. Molecular cloning, expression, and sequence similarity with histidine protein kinases | journal = The Journal of Biological Chemistry | volume = 267 | issue = 19 | pages = 13127–30 | date = Jul 1992 | doi = 10.1016/S0021-9258(18)42179-5 | pmid = 1377677 | doi-access = free }}</ref><ref name="pmid15302860">{{저널 인용| vauthors = Muller EA, Danner DJ | title = Tissue-specific translation of murine branched-chain alpha-ketoacid dehydrogenase kinase mRNA is dependent upon an upstream open reading frame in the 5'-untranslated region | journal = The Journal of Biological Chemistry | volume = 279 | issue = 43 | pages = 44645–55 | date = Oct 2004 | pmid = 15302860 | doi = 10.1074/jbc.M406550200 | doi-access = free }}</ref> 이 효소의 비정상적인 활성은 종종 [[단풍시럽뇨병]] 및 [[악액질]]과 같은 질병으로 이어진다.

== 같이 보기 ==
* [[가지사슬 아미노산]]
* [[가지사슬 케토산 탈수소효소 키네이스 결핍증]]

== 각주 ==
{{각주|33em}}

== 더 읽을거리 ==
{{참고 자료 시작|33em}}
* {{저널 인용| vauthors = Popov KM, Zhao Y, Shimomura Y, Kuntz MJ, Harris RA | title = Branched-chain alpha-ketoacid dehydrogenase kinase. Molecular cloning, expression, and sequence similarity with histidine protein kinases | journal = The Journal of Biological Chemistry | volume = 267 | issue = 19 | pages = 13127–30 | date = Jul 1992 | doi = 10.1016/S0021-9258(18)42179-5 | pmid = 1377677 | doi-access = free }}
* {{저널 인용| vauthors = Chang CF, Chou HT, Chuang JL, Chuang DT, Huang TH | title = Solution structure and dynamics of the lipoic acid-bearing domain of human mitochondrial branched-chain alpha-keto acid dehydrogenase complex | journal = The Journal of Biological Chemistry | volume = 277 | issue = 18 | pages = 15865–73 | date = May 2002 | pmid = 11839747 | doi = 10.1074/jbc.M110952200 | doi-access = free }}
* {{저널 인용| vauthors = Xu YC, Wu RF, Gu Y, Yang YS, Yang MC, Nwariaku FE, Terada LS | title = Involvement of TRAF4 in oxidative activation of c-Jun N-terminal kinase | journal = The Journal of Biological Chemistry | volume = 277 | issue = 31 | pages = 28051–7 | date = Aug 2002 | pmid = 12023963 | doi = 10.1074/jbc.M202665200 | doi-access = free }}
* {{저널 인용| vauthors = Starcevic M, Dell'Angelica EC | title = Identification of snapin and three novel proteins (BLOS1, BLOS2, and BLOS3/reduced pigmentation) as subunits of biogenesis of lysosome-related organelles complex-1 (BLOC-1) | journal = The Journal of Biological Chemistry | volume = 279 | issue = 27 | pages = 28393–401 | date = Jul 2004 | pmid = 15102850 | doi = 10.1074/jbc.M402513200 | doi-access = free }}
* {{저널 인용| vauthors = Wynn RM, Kato M, Machius M, Chuang JL, Li J, Tomchick DR, Chuang DT | title = Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation | journal = Structure | volume = 12 | issue = 12 | pages = 2185–96 | date = Dec 2004 | pmid = 15576032 | doi = 10.1016/j.str.2004.09.013 | doi-access = free }}
* {{저널 인용| vauthors = Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M | title = Towards a proteome-scale map of the human protein-protein interaction network | journal = Nature | volume = 437 | issue = 7062 | pages = 1173–8 | date = Oct 2005 | pmid = 16189514 | doi = 10.1038/nature04209 | bibcode = 2005Natur.437.1173R | s2cid = 4427026 }}
* {{저널 인용| vauthors = Ewing RM, Chu P, Elisma F, Li H, Taylor P, Climie S, McBroom-Cerajewski L, Robinson MD, O'Connor L, Li M, Taylor R, Dharsee M, Ho Y, Heilbut A, Moore L, Zhang S, Ornatsky O, Bukhman YV, Ethier M, Sheng Y, Vasilescu J, Abu-Farha M, Lambert JP, Duewel HS, Stewart II, Kuehl B, Hogue K, Colwill K, Gladwish K, Muskat B, Kinach R, Adams SL, Moran MF, Morin GB, Topaloglou T, Figeys D | title = Large-scale mapping of human protein-protein interactions by mass spectrometry | journal = Molecular Systems Biology | volume = 3 | issue = 1 | pages = 89 | year = 2007 | pmid = 17353931 | pmc = 1847948 | doi = 10.1038/msb4100134 }}
{{참고 자료 끝}}

== 외부 링크 ==
* {{UCSC genome browser|BCKDK}}
* {{UCSC gene details|BCKDK}}

{{효소}}
{{포털|생물학}}

[[분류:EC 2.7.11]]
[[분류:인간 16번 염색체상 유전자]]