BCKDHA Rabbit polyclonal antibody

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£151.00

SKU:: A9806
Zusätzliche Namen:: BCKDE1A|BCKDHA|MSU|MSUD1|OVD1A
Anwendung:: ELISA, WB, IF, ICC
Molekulargewicht:: 50kDa
Spezies-Reaktivität:: Human
Aufreinigung:: Affinity Purified
Lagerbedingungen:: -20[o]C Avoid freeze/thaw cycles.
Hersteller:: Abclonal
Host:: Rabbit
Reaktivitäten:: Human, Mouse, Rat
Immunogen:: Recombinant protein (or fragment).This information is considered to be commercially sensitive.
Formulierung:: Unmodified
Sequenz:: SEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHPISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK
Uniprot:: P12694
Synonyme:: 2-oxoisovalerate dehydrogenase (lipoamide);2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;BCKDE1A;BCKDH E1-alpha;branched chain keto acid dehydrogenase E1 alpha protein;branched chain keto acid dehydrogenase E1, alpha polypeptide;branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;MSU;MSUD1;OVD1A
Weitere Details:: 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.

Versandbedingungen:: Blue Ice