BCKDHB Antibody - N-terminal region : FITC

Conjugation: FITC: Fluorescein Isothiocyanate

Description of Target: 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.

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

Molecular Weight: 23kDa

Peptide Sequence: Synthetic peptide located within the following region: GFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDN

Product Format: Liquid. Purified antibody supplied in 1x PBS buffer.

Protein Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial Ensembl ENSP00000443564 Ensembl ENSP00000358775

Protein Size: 218

Purification: Affinity Purified

• SKU: AVIARP58593_P050FITC
• Manufacturer: Aviva Systems Biology
• Manufacturer SKU: ARP58593_P050-FITC
• Green Labware: No
• Package Unit: 100μl
• Quantity Unit: STK
• Reactivity: Human, Rat (Rattus), Pig (Porcine), Rabbit, Dog (Canine), Cow (Bovine), Horse (Equine)
• Clonality: Polyclonal
• Application: Western Blotting
• Human Gene ID: 594
• Host: Rabbit