HADHA
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Official Full Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit -
Overview
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] -
Synonyms
HADHA;hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit;GBP;ECHA;HADH;LCEH;MTPA;LCHAD;TP-ALPHA;trifunctional enzyme subunit alpha, mitochondrial;3-oxoacyl-CoA thiolase;gastrin-binding protein;78 kDa gastrin-binding protein;long-chain 2-enoyl-CoA hydratase;long-chain-3-hydroxyacyl-CoA dehydrogenase;mitochondrial trifunctional enzyme, alpha subunit;mitochondrial trifunctional protein, alpha subunit;3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit;mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit;mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit;hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
Recombinant Proteins
- Human
- Rhesus macaque
- Rat
- Chicken
- E.coli
- Mouse
- E.coli
- Mammalian Cell
- HEK293
- Insect Cell
- GST
- His
- Non
- His&Fc&Avi
- His&Myc
Involved Pathway
HADHA involved in several pathways and played different roles in them. We selected most pathways HADHA participated on our site, such as Fatty acid elongation,Fatty acid degradation,Valine, leucine and isoleucine degradation, which may be useful for your reference. Also, other proteins which involved in the same pathway with HADHA were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Tryptophan metabolism | ACAT2,GCDHB,ALDH2,CYP1A,AOX4,CYP4F12,ALDH9A1A.1,TPH1,ANI_1_2390104,CYP2F1 |
| Metabolic pathways | GALNT5,KDSR,PPOX,CYP21A1,CYP2R1,HMGCS1,GPT,IMPDH1B,PIGO,PYGM |
| Biosynthesis of unsaturated fatty acids | FADS2,Scd2,PECR,PTPLA,HSD17B12,TECR,HSD17B12B,ACOT1,ACAA1A,ACOT2 |
| Butanoate metabolism | OXCT2A,EHHADH,ALDH5A1,HADH,ACSM2,ACADS,HMGCS2,OXCT2,BDH2,AACS |
| Propanoate metabolism | ALDH6A1,MLYCD,ACSS1,ACAT1,PCCB,LDHAL6B,ACSS2L,LDHBB,BCKDHB,ACACB |
| beta-Alanine metabolism | ALDH2.1,ALDH9A1B,HADHAA,ACADM,UPB1,ALDH9A1A.1,ALDH1B1,CARNS1,ALDH3B2,ALDH2.2 |
| Fatty acid metabolism | ELOVL6,Scd1,MECR,ACSL5,ACADSB,ELOVL2,ACSL1,ACADVL,OXSM,HADH |
| Valine, leucine and isoleucine degradation | OXCT2,ALDH2.2,ALDH6A1,HADHAA,AOX2P,MCCC2,OXCT2A,IVD,ALDH2,ACSF3 |
| Fatty acid degradation | ACADSB,ACSL1A,ACSL5,ADH1A,ADH1B,ADH4,GCDHB,HADHAB,ACSL4A,ACAT2 |
Protein Function
HADHA has several biochemical functions, for example, 3-hydroxyacyl-CoA dehydrogenase activity,NAD binding,acetyl-CoA C-acetyltransferase activity. Some of the functions are cooperated with other proteins, some of the functions could acted by HADHA itself. We selected most functions HADHA had, and list some proteins which have the same functions with HADHA. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| NAD binding | PARP1,MDH1,ME1,AHCY,NNT,IDH3A,NDUFS2,SORD,CYB5R3,GPD1C |
| enoyl-CoA hydratase activity | HADHAB,HADHAA,ECHS1,EHHADH,HADHB,AUH |
| long-chain-enoyl-CoA hydratase activity | HSD17B4 |
| protein complex binding | VDAC1,IGBP1,NCKAP1,NRAS,HSPD1,PTCH1,ANKRD32,NDUFA8,FLCN,YWHAB |
| acetyl-CoA C-acetyltransferase activity | ACAA1A,ACAT1,ACAT2 |
| fatty-acyl-CoA binding | ACOX2,ACBD4,ACAD8,ACBD3,SOAT1,ACOX3,ACADM,ACBD6,PECI,ACBD5B |
| protein binding | WDTC1,LRRFIP1,CTBP1,UCHL3,KCNB1,PSMB5,ALG13,MED20,LDB3,KCND3 |
| long-chain-3-hydroxyacyl-CoA dehydrogenase activity | HADHB |
| 3-hydroxyacyl-CoA dehydrogenase activity | HSD17B4,H2-KE6,HSD17B10,HADHAB,HADH,HADHAA,EHHADH,HSD17B8,HADHB |
Interacting Protein
HADHA has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with HADHA here. Most of them are supplied by our site. Hope this information will be useful for your research of HADHA.
GABARAP;MAP1LC3B;TRAF6;GABARAPL2;GABARAPL1;MAP1LC3A
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