abx025837

antibody from Abbexa

Targeting: BCKDHB

No application information provided.

Antibody Data

Product number

abx025837

Provider

Abbexa

Product name

2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody

Antibody type

Polyclonal

Description

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.

Reactivity

Human

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

80 µl

Storage

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.