BCKDHB Antibody

Product Specifications

Background

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.

NCBI Gene ID

594

Swiss Prot

P21953

Accession Number

P21953

Host

Rabbit

Reactivity

Human

Immunogen

This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Obesity, Signal Transduction

Purification

This antibody is purified through a protein A column, followed by peptide affinity purification.

Concentration

Batch dependent

Buffer

Supplied in PBS with 0.09% (W/V) sodium azide.

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Calculated Molecular Weight

43 kDa

Fragment

Rabbit Ig

Symbol

BCKDHB

NCBI Official Name

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

NCBI Organism

Homo sapiens

Background Reference 01

Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :

Background Reference 02

Gorzelany, K., et al. Turk. J. Pediatr. 51 (2) :97-102 (2009)

Background Reference 03

Quental, S., et al. Mol. Genet. Metab. 94 (2) :148-156 (2008)

Background Reference 04

Kang, H., et al. Fertil. Steril. 89 (3) :728-731 (2008)

Other Product Names

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB