[KD Validated] DBT Rabbit monoclonal antibody

Size

POA

SKU:: A25001
Additional Names:: [KD Validated] DBT|BCATE2|BCKAD-E2|BCKADE2|BCKDH-E2|BCOADC-E2|E2|E2B
Application:: ELISA, WB, IHC-P, IP
Molecular Weight:: 53 kDa
Species Reactivity:: Human
Purification:: Affinity Purified
Storage Conditions:: -20[o]C Avoid freeze/thaw cycles.
Supplier:: Abclonal
Host:: Rabbit
Reactivities:: Human, Mouse, Rat
Immunogen:: Recombinant protein (or fragment).This information is considered to be commercially sensitive.
Formulation:: Unmodified
Sequence:: GQVVQFKLSDIGEGIREVTVKEWYVKEGDTVSQFDSICEVQSDKASVTITSRYDGVIKKLYYNLDDIAYVGKPLVDIETEALKDSEEDVVETPAVSHDEHTHQEIKGRKTLATPAVRRLAMENNIKLSEVVGSGKDGRILKEDILNYLEKQTGAILPPSPKVEIMPPPPKPKDMTVPILVSKPPVF
Uniprot:: P11182
Synonyms:: 52 kDa mitochondrial autoantigen of primary biliary cirrhosis;BCATE2;BCKAD E2 subunit;BCKAD-E2;BCKADE2;BCKDH-E2;BCOADC-E2;branched chain 2-oxo-acid dehydrogenase complex component E2;branched chain acyltransferase, E2 component;branched-chain alpha-keto acid dehydrogenase complex component E2;dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex;Dihydrolipoamide branched chain transacylase;dihydrolipoyl transacylase;dihydrolipoyllysine-residue (2-methylpropanoyl)transferase;E2;E2 component of branched chain alpha-keto acid dehydrogenase complex;E2B;lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial;lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex;mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b)
Extra Details:: 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.

Shipping Conditions:: Blue Ice