BCKDHB antibody (N-Term)

Catalog No. ABIN2787755

This Rabbit Polyclonal antibody specifically detects BCKDHB in WB. It exhibits reactivity toward Human, Rat, Cow, Dog, Horse, Pig and Rabbit.

Quick Overview for BCKDHB antibody (N-Term) (ABIN2787755)

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

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

Host: Rabbit

Clonality: Polyclonal

Conjugate: This BCKDHB antibody is un-conjugated

Application: Western Blotting (WB)

Product Details anti-BCKDHB Antibody

Binding Specificity: N-Term

Purpose: BCKDHB Antibody - N-terminal region

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

Application Notes: Optimal working dilution should be determined by the investigator.

Comment:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP58593-100UG

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: 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: prevent freeze-thaw cycles

Storage: 4 °C,-20 °C

Storage Comment: For short term use, store at 2-8C 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

Background:

Background Information: 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.

Gene Name: branched chain keto acid dehydrogenase E1, beta polypeptide

Alternative Symbols: E1B, BCKDE1B, BCKDH E1-beta

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

Molecular Weight: 23kDa

Gene ID: 594

UniProt: Q5T2J3