BCKDHB Antilichaam - N-terminalgebied: FITC (ARP58593 P050-FITC)

Branched chain keto acid dehydrogenase E1, beta polypeptide

E1B, BCKDE1B, BCKDH E1-beta

594

Q5T2J3

Human, Rat, Cow, Dog, Horse, Pig, Rabbit

The immunogen is a synthetic peptide directed towards the N-terminal region of Human BCKDHB

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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.

CUL3; BCKDHA

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 93%; Dog: 93%; Horse: 93%; Human: 100%; Pig: 86%; Rabbit: 93%; Rat: 79%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

23kDa

Wet Ice

218

BCKDHB

BCKDHB

Rabbit

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial Ensembl ENSP00000443564 Ensembl ENSP00000358775

GFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDN