BCKDHB antibody (N-Term)

Catalog No. ABIN2787755

Product Details anti-BCKDHB Antibody

Target: BCKDHB (Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB))

Binding Specificity: N-Term

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

Host: Rabbit

Clonality: Polyclonal

Conjugate: This BCKDHB antibody is un-conjugated

Application: Western Blotting (WB)

Sequence: GFLHPAATVE DAAQRRQVAH FTFQPDPEPR EYGQTQKMNL FQSVTSALDN

Predicted Reactivity: Cow: 93%, Dog: 93%, Horse: 93%, Human: 100%, Pig: 86%, Rabbit: 93%, Rat: 79%

Characteristics: This is a rabbit polyclonal antibody against BCKDHB. It was validated on Western Blot.

Purification: Affinity Purified

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

Application Details

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: Avoid repeat freeze-thaw cycles.

Storage: -20 °C

Storage Comment: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

Target Details for BCKDHB

Target: BCKDHB (Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB))

Alternative Name: BCKDHB (BCKDHB Products)

Synonyms: zgc:158777 antibody, BCKDHB antibody, E1B antibody, dJ279A18.1 antibody, branched chain keto acid dehydrogenase E1, beta polypeptide antibody, branched chain keto acid dehydrogenase E1 subunit beta antibody, branched chain keto acid dehydrogenase E1, beta polypeptide S homeolog antibody, branched chain ketoacid dehydrogenase E1, beta polypeptide antibody, BCKDHB antibody, bckdhb antibody, bckdhb.S antibody, Bckdhb antibody

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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.
Protein Interaction Partner: CUL3, BCKDHA,
Protein Size: 218

Molecular Weight: 23 kDa

Gene ID: 594

UniProt: Q5T2J3