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DBT Full-Length MS Protein Standard

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Product Description


NameDBT Full-Length MS Protein Standard
Cat#CPFL301998
Protein Namedihydrolipoamide branched chain transacylase E2
SymbolDBT
SynonymsBCATE2; BCKAD-E2; BCKADE2; BCOADC-E2; E2; E2B
AccessionNM_001918
Cytogenetic1p31
Amino Acid Labeled[U- 13C6, 15N4]-L-Arg and [U- 13C6, 15N2]-L-Lys
DescriptionDBT Full-Length MS Protein Standard (NP_001909), Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine, was produced in human 293 cells (HEK293) with fully chemically defined cell culture medium to obtain incorporation efficiency at Creative-Proteomics. The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.

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