HADHB

線粒體三功能酶β亞基（英語：Trifunctional enzyme subunit beta, mitochondrial，TP-beta）也被稱為3-ketoacyl-CoA thiolase、acetyl-CoA acyltransferase或beta-ketothiolase 是一種由人類基因 HADHB 所編碼的酶^[5]。

HADHB
識別號
別名	HADHB；, ECHB, MSTP029, MTPB, TP-BETA, hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit, hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta
外部ID	OMIM：143450 MGI：2136381 HomoloGene：153 GeneCards：HADHB
基因位置（人類） 染色體 2號染色體[1] 基因座 2p23.3 起始 26,243,170 bp[1] 終止 26,290,465 bp[1]
基因位置（小鼠） 染色體 小鼠5號染色體[2] 基因座 5|5 B1 起始 30,360,246 bp[2] 終止 30,389,591 bp[2]
基因本體 分子功能 • 轉移酶活性 • acyltransferase activity, transferring groups other than amino-acyl groups • enoyl-CoA hydratase activity • long-chain-3-hydroxyacyl-CoA dehydrogenase activity • 血漿蛋白結合 • 催化活性 • acyltransferase activity • 3-hydroxyacyl-CoA dehydrogenase activity • RNA binding • acetyl-CoA C-acyltransferase activity • long-chain-enoyl-CoA hydratase activity 細胞組分 • 膜 • mitochondrial outer membrane • 內質網 • mitochondrial inner membrane • mitochondrial envelope • 線粒體擬核 • 外排體 • 線粒體 生物學過程 • 脂質代謝 • 脂肪酸代謝 • 代謝 • cardiolipin acyl-chain remodeling • fatty acid beta-oxidation Sources:Amigo / QuickGO
直系同源
物種	人類	小鼠
Entrez	3032	231086
Ensembl	ENSG00000138029	ENSMUSG00000059447
UniProt	P55084	Q99JY0
mRNA​序列	NM_000183 ​NM_001281512 ​NM_001281513	NM_145558 ​NM_001289798 ​NM_001289799
蛋白序列	NP_000174 ​NP_001268441 ​NP_001268442	NP_001276727 ​NP_001276728 ​NP_663533
基因位置​（UCSC）	Chr 2: 26.24 – 26.29 Mb	Chr 5: 30.36 – 30.39 Mb
PubMed​查找	[3]	[4]
維基數據
檢視/編輯人類 檢視/編輯小鼠

HADHB是線粒體三功能蛋白的一個亞基，並具有硫解酶（英語：thiolase）活性。

結構

HADHB基因位於2號染色體的2p23段^[5]，包括17個外顯子，蛋白分子量為51.2 kDa，包括474個氨基酸殘基^[6][7]。

功能

參考文獻

1. GRCh38: Ensembl release 89: ENSG00000138029 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000059447 - Ensembl, May 2017
3. Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.
4. Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.
5. Entrez Gene: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein). （原始內容存檔於2009-10-19）.
6. ]Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P. Integration of cardiac proteome biology and medicine by a specialized knowledgebase. Circulation Research. October 2013, 113 (9): 1043–53. PMC 4076475 . PMID 23965338. doi:10.1161/CIRCRESAHA.113.301151.
7. Trifunctional enzyme subunit beta, mitochondrial. Cardiac Organellar Protein Atlas Knowledgebase (COPaKB). [2019-07-07]. （原始內容存檔於2016-03-04）.

延伸閱讀

• Wang R, Yang Z, Zhu JM; et al. [Screening for G1528C mutation in mitochondrial trifunctional protein gene in pregnant women with severe preeclampsia and new born infant].. Zhonghua Fu Chan Ke Za Zhi. 2006, 41 (10): 672–5. PMID 17199921. 引文格式1維護：顯式使用等標籤 (link)
• Aboulaich N, Vainonen JP, StrÃ¥lfors P, Vener AV. Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes.. Biochem. J. 2004, 383 (Pt 2): 237–48. PMC 1134064 . PMID 15242332. doi:10.1042/BJ20040647.
• Adams DJ, Beveridge DJ, van der Weyden L; et al. HADHB, HuR, and CP1 bind to the distal 3'-untranslated region of human renin mRNA and differentially modulate renin expression.. J. Biol. Chem. 2003, 278 (45): 44894–903. PMID 12933794. doi:10.1074/jbc.M307782200. 引文格式1維護：顯式使用等標籤 (link)
• Spiekerkoetter U, Khuchua Z, Yue Z; et al. General mitochondrial trifunctional protein (TFP) deficiency as a result of either alpha- or beta-subunit mutations exhibits similar phenotypes because mutations in either subunit alter TFP complex expression and subunit turnover.. Pediatr. Res. 2004, 55 (2): 190–6. PMID 14630990. doi:10.1203/01.PDR.0000103931.80055.06. 引文格式1維護：顯式使用等標籤 (link)
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• Middleton B. The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA thiolase.. Biochem. Soc. Trans. 1994, 22 (2): 427–31. PMID 7958339.
• Zhao Y, Meng XM, Wei YJ; et al. Cloning and characterization of a novel cardiac-specific kinase that interacts specifically with cardiac troponin I.. J. Mol. Med. 2003, 81 (5): 297–304. PMID 12721663. doi:10.1007/s00109-003-0427-x. 引文格式1維護：顯式使用等標籤 (link)
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• Purevsuren J, Fukao T, Hasegawa Y; et al. Clinical and molecular aspects of Japanese patients with mitochondrial trifunctional protein deficiency.. Mol. Genet. Metab. 2009, 98 (4): 372–7. PMID 19699128. doi:10.1016/j.ymgme.2009.07.011. 引文格式1維護：顯式使用等標籤 (link)
• Hillier LW, Graves TA, Fulton RS; et al. Generation and annotation of the DNA sequences of human chromosomes 2 and 4.. Nature. 2005, 434 (7034): 724–31. PMID 15815621. doi:10.1038/nature03466. 引文格式1維護：顯式使用等標籤 (link)
• Spiekerkoetter U, Sun B, Khuchua Z; et al. Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations.. Hum. Mutat. 2003, 21 (6): 598–607. PMID 12754706. doi:10.1002/humu.10211. 引文格式1維護：顯式使用等標籤 (link)
• Gerhard DS, Wagner L, Feingold EA; et al. The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).. Genome Res. 2004, 14 (10B): 2121–7. PMC 528928 . PMID 15489334. doi:10.1101/gr.2596504. 引文格式1維護：顯式使用等標籤 (link)
• Ota T, Suzuki Y, Nishikawa T; et al. Complete sequencing and characterization of 21,243 full-length human cDNAs.. Nat. Genet. 2004, 36 (1): 40–5. PMID 14702039. doi:10.1038/ng1285. 引文格式1維護：顯式使用等標籤 (link)
• Fould B, Garlatti V, Neumann E; et al. Structural and functional characterization of the recombinant human mitochondrial trifunctional protein.. Biochemistry. 2010, 49 (39): 8608–17. PMID 20825197. doi:10.1021/bi100742w. 引文格式1維護：顯式使用等標籤 (link)
• Strausberg RL, Feingold EA, Grouse LH; et al. Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.. Proc. Natl. Acad. Sci. U.S.A. 2002, 99 (26): 16899–903. PMC 139241 . PMID 12477932. doi:10.1073/pnas.242603899. 引文格式1維護：顯式使用等標籤 (link)
• Ibdah JA, Tein I, Dionisi-Vici C; et al. Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype-phenotype correlation.. J. Clin. Invest. 1998, 102 (6): 1193–9. PMID 9739053. doi:10.1172/JCI2091. 引文格式1維護：顯式使用等標籤 (link)
• Gevaert K, Goethals M, Martens L; et al. Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides.. Nat. Biotechnol. 2003, 21 (5): 566–9. PMID 12665801. doi:10.1038/nbt810. 引文格式1維護：顯式使用等標籤 (link)
• Hendrickson SL, Lautenberger JA, Chinn LW; et al. Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.. PLoS ONE. 2010, 5 (9): e12862. PMC 2943476 . PMID 20877624. doi:10.1371/journal.pone.0012862. 引文格式1維護：顯式使用等標籤 (link)
• Frackowiak J, Mazur-Kolecka B, Kaczmarski W, Dickson D. Deposition of Alzheimer's vascular amyloid-beta is associated with decreased expression of brain L-3-hydroxyacyl-coenzyme A dehydrogenase (ERAB).. Brain Res. 2001, 907 (1-2): 44–53. PMID 11430884. doi:10.1016/S0006-8993(01)02497-0.

外部連結

• 醫學主題詞表（MeSH）：HADHB+protein,+human

HADHB引用了美國國家醫學圖書館提供的資料，這些資料屬於公共領域。