Gene/Proteome Database (LMPD)

LMPD ID
LMP001501
Gene ID
3030
Species
Homo sapiens (Human)
Gene Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
Gene Symbol
HADHA
Synonyms
ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA
Chromosome
2
Map Location
2p23
Summary
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]
Orthologs
View orthologs and multiple alignments for HADHA

Proteins

trifunctional enzyme subunit alpha, mitochondrial precursor
Refseq ID NP_000173
Protein GI 20127408
UniProt ID P40939
mRNA ID NM_000182
Length 763
RefSeq Status REVIEWED
MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALLTRTHINYGVKGDVAVVRINSPNSKVNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLSQEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALPGAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYLEEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPAPLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKDVKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSFERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALPISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVAEDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLKLPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ
transit_peptide: 1..36 calculated_mol_wt: 4008 peptide sequence: MVACRAIGILSRFSAFRILRSRGYICRNFTGSSALL mat_peptide: 37..763 product: trifunctional enzyme subunit alpha, mitochondrial calculated_mol_wt: 79010 peptide sequence: TRTHINYGVKGDVAVVRINSPNSKVNTLSKELHSEFSEVMNEIWASDQIRSAVLISSKPGCFIAGADINMLAACKTLQEVTQLSQEAQRIVEKLEKSTKPIVAAINGSCLGGGLEVAISCQYRIATKDRKTVLGTPEVLLGALPGAGGTQRLPKMVGVPAALDMMLTGRSIRADRAKKMGLVDQLVEPLGPGLKPPEERTIEYLEEVAITFAKGLADKKISPKRDKGLVEKLTAYAMTIPFVRQQVYKKVEEKVRKQTKGLYPAPLKIIDVVKTGIEQGSDAGYLCESQKFGELVMTKESKALMGLYHGQVLCKKNKFGAPQKDVKHLAILGAGLMGAGIAQVSVDKGLKTILKDATLTALDRGQQQVFKGLNDKVKKKALTSFERDSIFSNLTGQLDYQGFEKADMVIEAVFEDLSLKHRVLKEVEAVIPDHCIFASNTSALPISEIAAVSKRPEKVIGMHYFSPVDKMQLLEIITTEKTSKDTSASAVAVGLKQGKVIIVVKDGPGFYTTRCLAPMMSEVIRILQEGVDPKKLDSLTTSFGFPVGAATLVDEVGVDVAKHVAEDLGKVFGERFGGGNPELLTQMVSKGFLGRKSGKGFYIYQEGVKRKDLNSDMDSILASLKLPPKSEVSSDEDIQFRLVTRFVNEAVMCLQEGILATPAEGDIGAVFGLGFPPCLGGPFRFVDLYGAQKIVDRLKKYEAAYGKQFTPCQLLADHANSPNKKFYQ

Gene Information

Entrez Gene ID
3030
Gene Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
Gene Symbol
HADHA
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0016507 IEA:InterPro C mitochondrial fatty acid beta-oxidation multienzyme complex
GO:0005743 IEA:Ensembl C mitochondrial inner membrane
GO:0005739 IDA:HPA C mitochondrion
GO:0051287 IEA:Ensembl F NAD binding
GO:0003988 IEA:Ensembl F acetyl-CoA C-acyltransferase activity
GO:0004300 IEA:InterPro F enoyl-CoA hydratase activity
GO:0000062 IEA:Ensembl F fatty-acyl-CoA binding
GO:0016509 IEA:Ensembl F long-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO:0016508 IEA:Ensembl F long-chain-enoyl-CoA hydratase activity
GO:0006635 IEA:InterPro P fatty acid beta-oxidation
GO:0042493 IEA:Ensembl P response to drug
GO:0032868 IEA:Ensembl P response to insulin

KEGG Pathway Links

KEGG Pathway ID Description
hsa01200 Carbon metabolism

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_121006 Acyl chain remodeling of CL

Domain Information

InterPro Annotations

Accession Description
IPR006108 3-hydroxyacyl-CoA dehydrogenase, C-terminal
IPR006176 3-hydroxyacyl-CoA dehydrogenase, NAD binding
IPR006180 3-hydroxyacyl-CoA dehydrogenase, conserved site
IPR008927 6-phosphogluconate dehydrogenase, C-terminal-like
IPR029045 ClpP/crotonase-like domain
IPR001753 Crotonase_core_superfam
IPR013328 Dehydrogenase, multihelical
IPR018376 Enoyl-CoA hydratase/isomerase, conserved site
IPR012803 Fatty acid oxidation complex, alpha subunit, mitochondrial
IPR016040 NAD(P)-binding domain

UniProt Annotations

Entry Information

Gene Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
Protein Entry
ECHA_HUMAN
UniProt ID
P40939
Species
Human

Identical and Related Proteins

Unique RefSeq proteins for LMP001501 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
20127408 RefSeq NP_000173 763 trifunctional enzyme subunit alpha, mitochondrial precursor

Identical Sequences to LMP001501 proteins

Reference Database Accession Length Protein Name
GI:20127408 GenBank ADT49081.1 763 Sequence 1098 from patent US 7842467
GI:20127408 GenBank ADT49082.1 763 Sequence 1099 from patent US 7842467
GI:20127408 GenBank ADU87643.1 763 epididymis tissue sperm binding protein Li 14m [Homo sapiens]
GI:20127408 GenBank AEN35812.1 763 Sequence 1740 from patent US 7998689
GI:20127408 GenBank AEN35813.1 763 Sequence 1741 from patent US 7998689
GI:20127408 GenBank AIC48918.1 763 HADHA, partial [synthetic construct]

Related Sequences to LMP001501 proteins

Reference Database Accession Length Protein Name
GI:20127408 DBBJ BAA03941.1 763 enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase alpha-subunit of trifunctional protein [Homo sapiens]
GI:20127408 GenBank ACM82262.1 772 Sequence 7760 from patent US 6812339
GI:20127408 GenBank JAA18923.1 763 hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Pan troglodytes]
GI:20127408 GenBank JAA32530.1 763 hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Pan troglodytes]
GI:20127408 GenBank JAA38320.1 763 hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Pan troglodytes]
GI:20127408 RefSeq XP_003827103.1 763 PREDICTED: trifunctional enzyme subunit alpha, mitochondrial [Pan paniscus]