ECHA rabbit pAb

• Background :

  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]

• Description :

  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 :

  P40939

• Swiss Prot :

  P40939

• Reactivity :

  Human; Mouse; Rat

• Immunogen :

  Synthesized peptide derived from human ECHA AA range: 276-326

• Clonality :

  Polyclonal

• Source :

  Rabbit

• Applications :

  WB

• Concentration :

  1 mg/ml

• Dilution :

  WB 1:500-2000

• Molecular Weight :

  85kD

• Storage Conditions :

  -20°C/1 year

• Observed Molecular Weight :

  85kD

• Fragment :

  IgG

• Subcellular Location :

  Mitochondrion . Mitochondrion inner membrane . Protein stability and association with mitochondrion inner membrane do not require HADHB. .

• Other Product Names :

  Trifunctional enzyme subunit alpha, mitochondrial (78 kDa gastrin-binding protein) (TP-alpha) [Includes: Long-chain enoyl-CoA hydratase (EC 4.2.1.17) ; Long chain 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.211) ]

• Gene ID (Human) :

  3030