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Gene: HADHA (hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha) Homo sapiens
Symbol: HADHA
Name: hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
Description: Predicted to have enoyl-CoA hydratase activity and long-chain-3-hydroxyacyl-CoA dehydrogenase activity. Involved in cardiolipin acyl-chain remodeling. Localizes to the mitochondrial nucleoid. Implicated in fatty liver disease and mitochondrial trifunctional protein deficiency. Biomarker of Alzheimer's disease.
Type: protein-coding
RefSeq Status: REVIEWED
Also known as: 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit; 3-oxoacyl-CoA thiolase; 78 kDa gastrin-binding protein; ECHA; gastrin-binding protein; GBP; HADH; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; hydroxyacyl-coenzyme a dehydrogenase/3-ketoacyl-coenzyme a hiolase/enoyl-coenzyme a hydratase (trifunctional protein), alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; LCEH; LCHAD; long-chain 2-enoyl-CoA hydratase; long-chain-3-hydroxyacyl-CoA dehydrogenase; MGC1728; mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit; mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit; mitochondrial trifunctional enzyme, alpha subunit; mitochondrial trifunctional protein, alpha subunit; monolysocardiolipin acyltransferase; MTPA; TP-ALPHA; trifunctional enzyme subunit alpha, mitochondrial
Related Pseudogenes: HADHAP1   HADHAP2  
Allele / Splice: See ClinVar data
Latest Assembly: GRCh38 - Human Genome Assembly GRCh38
Human AssemblyChrPosition (strand)SourceGenome Browsers
GRCh38226,190,635 - 26,244,632 (-)NCBIGRCh38GRCh38hg38GRCh38
GRCh37226,413,504 - 26,467,665 (-)NCBIGRCh37GRCh37hg19GRCh37
Build 36226,267,008 - 26,321,098 (-)NCBINCBI36hg18NCBI36
Build 34226,325,159 - 26,379,140NCBI
Celera226,253,733 - 26,307,825 (-)NCBI
Cytogenetic Map2p23.3NCBI
HuRef226,149,287 - 26,203,382 (-)NCBIHuRef
CHM1_1226,343,424 - 26,397,498 (-)NCBICHM1_1
JBrowse: View Region in Genome Browser (JBrowse)

Disease Annotations
Gene-Chemical Interaction Annotations
Gene Ontology Annotations
Molecular Pathway Annotations
Phenotype Annotations
References - curated
References - uncurated


Comparative Map Data
Position Markers
miRNA Target Status


Nucleotide Sequences
Protein Sequences
Clinical Variants

Additional Information

External Database Links
Nomenclature History
More on HADHA
Alliance Gene
Ensembl Gene
JBrowse: hg19 hg38
HGNC Report
NCBI Genome Data Viewer

CRRD Object Information
CRRD ID: 733877
Created: 2004-01-12
Species: Homo sapiens
Last Modified: 2019-11-05
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.