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| Cat.No | ACP21639 | Target Name | BCKDK |
|---|---|---|---|
| Target Synonyms | [3 methyl 2 oxobutanoate dehydrogenase [lipoamide]] kinase | Form | Lyophilized powder |
| Expression System | Custom Production. Please inquire and provide the desire expression system. | Expression Range | 31-412 |
| 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 | O14874 |
|---|
Uniprot Id
O14874
Target Species
Human
Target Name
BCKDK
Target Full Name
Branched-chain alpha-ketoacid dehydrogenase kinase
Target Function
Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex.
Target Involvement
Branched-chain ketoacid dehydrogenase kinase deficiency (BCKDKD)
Target Subcellular Location
Mitochondrion matrix. Mitochondrion.
Target Protein Families
PDK/BCKDK protein kinase family
Target Tissue Specificity
Ubiquitous.
Target Synonyms
[3 methyl 2 oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial ; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase; BCKD kinase ; BCKD-kinase; BCKD_HUMAN; BCKDHKIN; Bckdk; BCKDKD; BDK; Branched chain alpha keto acid dehydrogenase kinase; Branched chain ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; mitochondrial
Target Background
The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene.
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