BCKDHB, Rabbit, Polyclonal Antibody, Abnova™

Rabbit polyclonal antibody raised against recombinant BCKDHB.

Supplier:  Abnova Corporation PAB22782

Catalog No. 89-124-488

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. Alternative splicing at this locus results in transcript variants with different 3' noncoding regions, but encoding the same isoform. [provided by RefSeq]

Sequence: QVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRD

Specifications

• Antigen: BCKDHB
• Classification: Polyclonal
• Description: Rabbit polyclonal antibody raised against recombinant BCKDHB.
• Formulation: In PBS, pH 7.5 (40% glycerol, 0.02% sodium azide)
• Gene Accession No.: P21953
• Gene Symbols: BCKDHB
• Immunogen: Recombinant protein corresponding to amino acids of human BCKDHB.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Form: Liquid

• Applications: Immunohistochemistry (PFA fixed), Western Blot
• Conjugate: Unconjugated
• Dilution: Immunohistochemistry (1:20-1:50) Western Blot (1:250-1:500) The optimal working dilution should be determined by the end user.
• Gene: BCKDHB
• Gene Alias: E1B/FLJ17880/dJ279A18.1
• Host Species: Rabbit
• Purification Method: Antigen affinity purification
• Regulatory Status: RUO
• Gene ID (Entrez): 594
• Content And Storage: Store at 4°C. For long term storage store at -20°C.
  Aliquot to avoid repeated freezing and thawing.
• Isotype: IgG

For Research Use Only