NP_000173.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
Observed: 83 kDa Predicted: 83 kDa
NCBI Official Full Name
trifunctional enzyme subunit alpha, mitochondrial
NCBI Official Synonym Full Names
hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
NCBI Official Synonym Symbols
GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA [Similar Products]
NCBI Protein Information
trifunctional enzyme subunit alpha, mitochondrial
UniProt Protein Name
Trifunctional enzyme subunit alpha, mitochondrial
UniProt Synonym Protein Names
78 kDa gastrin-binding protein; TP-alphaIncluding the following 2 domains:Long-chain enoyl-CoA hydratase (EC:4.2.1.17)Long chain 3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.211)
UniProt Synonym Gene Names
NCBI Summary for HADHA
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]
UniProt Comments for HADHA
Bifunctional subunit.
Research Articles on HADHA
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Products associated with anti-HADHA antibody
Pathways associated with anti-HADHA antibody
Diseases associated with anti-HADHA antibody
Organs/Tissues associated with anti-HADHA antibody
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