BCKDHB Proteins
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.
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Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) (AA 51-392) protein (His tag) Protein
Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) (AA 49-390) protein (His tag) Protein
Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) (AA 1-392) protein (GST tag) Protein
Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) (Transcript Variant 1) protein (Myc-DYKDDDDK Tag) Protein
Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) (AA 49-390) protein (His tag) Protein
Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB) (AA 51-392) protein (His tag) Protein
BCKDHB Protein Profile
Protein 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.
Gene names and symbols associated with BCKDHB
- branched chain keto acid dehydrogenase E1, beta polypeptide (BCKDHB) antibody
- branched chain keto acid dehydrogenase E1 subunit beta (BCKDHB) antibody
- branched chain keto acid dehydrogenase E1, beta polypeptide (bckdhb) antibody
- branched chain keto acid dehydrogenase E1, beta polypeptide S homeolog (bckdhb.S) antibody
- branched chain keto acid dehydrogenase E1 subunit beta (Bckdhb) antibody
- branched chain ketoacid dehydrogenase E1, beta polypeptide (Bckdhb) antibody
- BCKDHB antibody
- dJ279A18.1 antibody
- E1B antibody
- zgc:158777 antibody
Protein level used designations for BCKDHB
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial , branched chain keto acid dehydrogenase E1, beta polypeptide (maple syrup urine disease) , mitochondrial branched-chain alpha-keto acid dehydrogenase E1-beta subunit , branched chain keto acid dehydrogenase E1, beta polypeptide , branched chain keto acid dehydrogenase E1 beta polypeptide , branched chain keto acid dehydrogenase E1 beta-like , 2-oxoisovalerate dehydrogenase beta subunit , BCKDE1B , BCKDH E1-beta , branched chain keto acid dehydrogenase E1 beta , branched-chain alpha-keto acid dehydrogenase E1 component beta chain , E1b-beta subunit of the branched-chain complex , branched chain alpha-ketoacid dehydrogenase E1-beta subunit
GENE ID | SPECIES |
---|---|
395375 | Gallus gallus |
462845 | Pan troglodytes |
474978 | Canis lupus familiaris |
569980 | Danio rerio |
100023799 | Monodelphis domestica |
100065300 | Equus caballus |
100137712 | Xenopus laevis |
100142669 | Sus scrofa |
100173401 | Pongo abelii |
100353682 | Oryctolagus cuniculus |
100597493 | Nomascus leucogenys |
29711 | Rattus norvegicus |
594 | Homo sapiens |
282150 | Bos taurus |
12040 | Mus musculus |
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