HADH Rabbit Polyclonal Antibody (R1411-4)

UNIPROT #

Q16836

PROTEIN NAME

HADH

SYNONYMS

3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit antibody;3 oxoacyl CoA thiolase antibody;78 kDa gastrin binding protein antibody;78 kDa gastrin-binding protein antibody;ECHA antibody;ECHA_HUMAN antibody;GBP antibody;HADH antibody;HADHA antibody;Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody;LCEH antibody;LCHAD antibody;Long chain 3-hydroxyacyl-CoA dehydrogenase antibody;Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit antibody;Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit antibody;Mitochondrial trifunctional enzyme alpha subunit antibody;Mitochondrial trifunctional protein alpha subunit antibody;MTPA antibody;Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit antibody;TP ALPHA antibody;TP-alpha antibody;Trifunctional enzyme subunit alpha mitochondrial precursor antibody

SEQUENCE SIMILARITIES

Belongs to the 3-hydroxyacyl-CoA dehydrogenase family.

TISSUE SPECIFICITY

Expressed in liver, kidney, pancreas, heart and skeletal muscle.

POST-TRANSLATIONAL MODIFICATION

Succinylation at Lys-81, adjacent to a coenzyme A binding site. Desuccinylated by SIRT5 (By similarity).

SUBCELLULAR LOCATION

Mitochondrion.

FUNCTION

Hydroxyacyl-Coenzyme A dehydrogenase also known as HADH is an enzyme which in humans is encoded by the HADH gene. This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. A deficiency is associated with 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.