BCKDHB
branched chain keto acid dehydrogenase E1, beta polypeptide
Sinónimos:
E1B, dJ279A18.1
Especie:
Human BCKDHB (594), Mouse Bckdhb (12040), Rat Bckdhb (29711), naked mole-rat Bckdhb (101725476), domestic guinea pig Bckdhb (100731951), sheep BCKDHB (101113939), Domestic Rabbit BCKDHB (100353682), Zebrafish bckdhb (569980), cow BCKDHB (282150), Horse BCKDHB (100065300) ...Más
ID UniProtKB:
ID del gen:
- Human(594) Summary: Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein 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 transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq, Jul 2008]
- Mouse(12040) branched chain ketoacid dehydrogenase E1, beta polypeptide
- Rat(29711) branched chain keto acid dehydrogenase E1, beta polypeptide
- naked mole-rat(101725476) branched chain keto acid dehydrogenase E1, beta polypeptide
- domestic guinea pig(100731951) branched chain keto acid dehydrogenase E1, beta polypeptide
- sheep(101113939) branched chain keto acid dehydrogenase E1, beta polypeptide
- Domestic Rabbit(100353682) branched chain keto acid dehydrogenase E1, beta polypeptide
- Zebrafish(569980) branched chain ketoacid dehydrogenase E1, beta polypeptide
- cow(282150) branched chain keto acid dehydrogenase E1, beta polypeptide
- Horse(100065300) branched chain keto acid dehydrogenase E1, beta polypeptide
- domestic cat(101080635) branched chain keto acid dehydrogenase E1, beta polypeptide
- dog(474978) branched chain keto acid dehydrogenase E1, beta polypeptide
- chicken(395375) branched chain keto acid dehydrogenase E1, beta polypeptide
Custom & Knockdown Gene Products
siRNA
Shop predesigned siRNA that are designed using the proprietary Rosetta Inpharmatics algorithm.
shRNA
Shop the largest, most validated shRNA collection and build your own clone sets.
Custom Gene Products
We offer a comprehensive portfolio of DNA oligos, RNA oligos and predesigned primers.
Antibodies
Referencia del producto
Descripción
Reactividad de especies
Aplicación
Anti-BCKDHB antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Reactividad de especies
human
Aplicación
immunofluorescence