abx025837

BCKDHB antibody from Abbexa
No application information provided.

Antibody data

Product number
abx025837
Provider
Abbexa
Product name
2-Oxoisovalerate Dehydrogenase Subunit Beta, Mitochondrial (BCKDHB) Antibody
Provider product page
Abbexa - abx025837
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.
Provider Type Product Number
- No reagents -