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BCKDHB


Official Full Name
branched chain keto acid dehydrogenase E1 subunit beta
Organism
Homo sapiens
Gene ID
594
Background
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. [provided by RefSeq, Jan 2016]
Synonyms
E1B; OVD1B; MSUD1B; BCKDE1B; BCKDH E1-beta

Cat.No. Product Name Price
SHH245830 shRNA set against Rat BCKDHB (NM_019267.1) Inquiry
SHG096601 shRNA set against Mouse Bckdhb(NM_199195.1) Inquiry
SHG096685 shRNA set against Human BCKDHB(NM_000056.3) Inquiry
SHH245822 shRNA set against Human BCKDHB (NM_183050.2) Inquiry
SHH245826 shRNA set against Mouse BCKDHB (NM_199195.1) Inquiry
SHW005168 shRNA set against Chicken BCKDHB (NM_204657) Inquiry
SHW011019 shRNA set against Danio rerio BCKDHB (NM_001080653) Inquiry
Cat.No. Product Name Price
CDFR011558 Rat Bckdhb cDNA Clone(NM_019267.1) Inquiry
MiUTR1M-02150 BCKDHB miRNA 3'UTR clone Inquiry
MiUTR3H-02477 BCKDHB miRNA 3'UTR clone Inquiry
CDCB158134 Human BCKDHB ORF clone (BC040139) Inquiry
CDCB166643 Chicken BCKDHB ORF Clone (NM_204657) Inquiry
CDCB172494 Danio rerio BCKDHB ORF Clone (NM_001080653) Inquiry
CDCB185706 Rabbit BCKDHB ORF clone (XM_002714546.2) Inquiry
CDCR034052 Mouse Bckdhb ORF clone (NM_199195.1) Inquiry
CDCR378561 Rat Bckdhb ORF Clone(NM_019267.1) Inquiry
CDCS405386 Human BCKDHB ORF Clone (BC040139) Inquiry
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