BC038948
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
69,154 Da
NCBI Official Full Name
Homo sapiens enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase, mRNA
NCBI Official Synonym Full Names
enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase
NCBI Official Synonym Symbols
LBP; ECHD; LBFP; PBFE; FRTS3; L-PBE [Similar Products]
NCBI Protein Information
peroxisomal bifunctional enzyme
UniProt Protein Name
Peroxisomal bifunctional enzyme
UniProt Synonym Gene Names
UniProt Entry Name
ECHP_HUMAN
NCBI Summary for EHHADH
The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
UniProt Comments for EHHADH
EHHADH: 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Other Amino Acids Metabolism - beta-alanine; Oxidoreductase; Isomerase; EC 1.1.1.35; Amino Acid Metabolism - lysine degradation; EC 4.2.1.17; Secondary Metabolites Metabolism - limonene and pinene degradation; Carbohydrate Metabolism - propanoate; Lipid Metabolism - fatty acid; EC 5.3.3.8; Amino Acid Metabolism - tryptophan; Carbohydrate Metabolism - butanoate
Chromosomal Location of Human Ortholog: 3q26.3-q28
Cellular Component: peroxisomal matrix; peroxisome
Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; enzyme binding; long-chain-enoyl-CoA hydratase activity; protein binding; receptor binding
Biological Process: cellular lipid metabolic process; internal protein amino acid acetylation
Disease: Fanconi Renotubular Syndrome 3
Research Articles on EHHADH
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Pathways associated with EHHADH cdna clone
Diseases associated with EHHADH cdna clone
Organs/Tissues associated with EHHADH cdna clone
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