BCKDHB Rabbit monoclonal antibody

Size

POA

SKU:: A24618
Additional Names:: BCKDE1B|BCKDH E1-beta|BCKDHB|E1B
Application:: ELISA, WB, IHC-P
Molecular Weight:: 37 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:: VAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
Uniprot:: P21953
Synonyms:: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial;BCKDE1B;BCKDH E1-beta;branched chain alpha-ketoacid dehydrogenase E1-beta subunit;branched chain keto acid dehydrogenase E1 beta;branched chain keto acid dehydrogenase E1, beta polypeptide;branched-chain alpha-keto acid dehydrogenase E1 component beta chain;E1B;E1b-beta subunit of the branched-chain complex;testis secretory sperm-binding protein Li 240mP
Extra Details:: This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.

Shipping Conditions:: Blue Ice