SAB1405509

Anti-BCKDHA antibody produced in mouse

purified immunoglobulin, buffered aqueous solution

• Synonym(s): BCKDE1A, FLJ45695, MSU, MSUD1, OVD1A
• UNSPSC Code: 12352203
• NACRES: NA.41

Properties

• biological source: mouse
• Quality Level: 100
• conjugate: unconjugated
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: polyclonal
• form: buffered aqueous solution
• mol wt: antigen ~50.5 kDa
• species reactivity: human
• technique(s): western blot: 1 μg/mL
• NCBI accession no.: NM_000709.2
• UniProt accession no.: P12694
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... BCKDHA(593)

Description

General description

The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene

Immunogen

BCKDHA (NP_000700.1, 1 a.a. ~ 445 a.a) full-length human protein.

Sequence
MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYGNISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK

Physical form

Solution in phosphate buffered saline, pH 7.4

Safety Information

• Storage Class Code: 10 - Combustible liquids
• WGK: WGK 1
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable