BCKDHB antibody (N-Term)

Catalog No. ABIN950620

This Rabbit Polyclonal antibody specifically detects BCKDHB in WB, FACS, IF, IHC (p) and EIA. It exhibits reactivity toward Human.

Quick Overview for BCKDHB antibody (N-Term) (ABIN950620)

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

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This BCKDHB antibody is un-conjugated

Application: Western Blotting (WB), Flow Cytometry (FACS), Immunofluorescence (IF), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Enzyme Immunoassay (EIA)

Product Details anti-BCKDHB Antibody

Binding Specificity: AA 44-74, N-Term

Specificity: This antibody reacts to BCKDHB.

Cross-Reactivity (Details): Species reactivity (tested):Human.

Purification: Affinity chromatography on Protein A

Immunogen: KLH conjugated synthetic peptide between 44-74 amino acids from the N-terminal region of human BCKDHB

Isotype: Ig Fraction

Application Details

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

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.25 mg/mL

Buffer: PBS containing 0.09 % (W/V) sodium azide as preservative

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 repeated freezing and thawing.

Storage: 4 °C/-20 °C

Storage Comment: Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

Target Details for BCKDHB

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

Alternative Name: BCKDHB

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.Synonyms: 2-oxoisovalerate dehydrogenase subunit beta, alpha-keto acid dehydrogenase, mitochondrial Branched-chain alpha-keto acid dehydrogenase E1 component beta chain

Gene ID: 594

NCBI Accession: NP_000047