BCKDHA antibody [HL2405]

Eine Lieferung in diese Region ist nicht möglich.

SKU:: GTX638622
Zusätzliche Namen:: branched chain keto acid dehydrogenase E1, alpha polypeptide , BCKDE1A , MSU , MSUD1 , OVD1A
Anwendung:: IHC, WB, IHC-P, IF, ICC
Konzentration:: 1 mg/ml
Physischer Zustand:: Liquid
Spezies-Reaktivität:: Human
Aufreinigung:: Protein A Purified
Lagerbedingungen:: -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
Hersteller:: Genetex
Host:: Rabbit
Reaktivitäten:: Fish, Human, Mouse
Buffer:: PBS, no preservatives.
Immunogen:: Recombinant fragment of human BCKDHA.
Klon:: HL2405
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.[provided by RefSeq, Sep 2009]

Versandbedingungen:: Blue Ice