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HADHA (基因名), Trifunctional enzyme subunit alpha, mitochondrial (蛋白名), echa_human.

产品名称:

Human HADHA/ Trifunctional enzyme subunit alpha, mitochondrial CLIA Kit
线粒体三功能酶亚基α

货号:

U14271h

商标:

EIAab^®

监管等级:

RUO

别名:

78 kDa gastrin-binding protein, Monolysocardiolipin acyltransferase, TP-alpha, HADH

检测方法:

CLIA

实验类型:

Sandwich

检测范围:

0.078-5ng/mL

特异性:

Natural and recombinant human Trifunctional enzyme subunit alpha, mitochondrial

样品类型:

Serum, plasma, tissue homogenates, cell culture supernates and other biological fluids

样品数据:

研究领域:

Cardiovascular

规格 & 价格:

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产品说明书

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通用注释

亚单元:

Heterotetramer of 2 alpha/HADHA and 2 beta/HADHB subunits; forms the mitochondrial trifunctional enzyme (PubMed:29915090, PubMed:30850536). Also purified as higher order heterooligomers including a 4 alpha/HADHA and 4 beta/HADHB heterooligomer which physiological significance remains unclear (PubMed:8163672, PubMed:29915090).

功能:

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed:8135828, PubMed:1550553, PubMed:29915090, PubMed:30850536). The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA (PubMed:29915090). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed:30850536). Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity (PubMed:8135828, PubMed:29915090, PubMed:30850536). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed:23152787). It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP (PubMed:23152787). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed:23152787).

亚细胞位置:

Mitochondrion Mitochondrion inner membrane Protein stability and association with mitochondrion inner membrane do not require HADHB.

数据库链接

UniGene:

Hs.516032

SMR:

P40939

STRING:

9606.ENSP00000370023

KEGG:

hsa:3030

MIM: