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| Cat.No | ACP23936 | Target Name | BCKDHB |
|---|---|---|---|
| Form | Lyophilized powder | Expression System | Custom Production. Please inquire and provide the desire expression system. |
| Expression Range | 51-392 | Protein Length | Full Length of Mature Protein |
| Purity | >85% (SDS-PAGE) | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P21953 |
|---|
Uniprot Id
P21953
Target Species
Human
Target Name
BCKDHB
Target Full Name
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Target Function
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Target Involvement
Maple syrup urine disease 1B (MSUD1B)
Target Subcellular Location
Mitochondrion matrix.
Target Synonyms
2 oxoisovalerate dehydrogenase subunit beta mitochondrial; 2-oxoisovalerate dehydrogenase subunit beta; mitochondrial; BCKDE1B; BCKDH E1-beta; BCKDHB; Branched chain alpha ketoacid dehydrogenase E1 beta subunit; Branched chain keto acid dehydrogenase E1 beta polypeptide ; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1; E1B; E1b beta subunit of the branched chain complex; FLJ17880; ODBB_HUMAN; RP1-279A18.1
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