Human Recombinant BCKDK protein, GST Tag (V2LY-0526-LY2308)

Name: Human Recombinant BCKDK protein, GST Tag
SKU: V2LY-0526-LY2308
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Expressed Host

Baculovirus-Insect Cells

Protein Species

Human

Tag

GST Tag

Protein Construction

This product is Human Recombinant BCKDK protein, GST Tag consist of Amino Acid: 30-end and predicts a molecular mass of 69 kDa.

Molecule Mass

69 kDa

Sequence

Amino Acid: 30-end

Species

Human

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity

Batch dependent.

Endotoxin

Please contact us for more information.

Format

Liquid

Buffer

Tris, NaCl, Glutathione, EDTA, DTT, PMSF, Glycerol

Preservative

None

Storage

Store product at -70°C. For optimal storage, aliquot target into smaller quantities after centrifugation and store at recommended temperature. For most favorable performance, avoid repeated handling and multiple freeze/thaw cycles.

More Infomation

Target

Full Name

Branched Chain Ketoacid Dehydrogenase Kinase

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.

Biological Process

Branched-chain amino acid catabolic process Source: HGNC-UCL
Cellular amino acid catabolic process Source: UniProtKB
Isoleucine catabolic process Source: Ensembl
Leucine catabolic process Source: Ensembl
Negative regulation of cellular amino acid metabolic process Source: Reactome
Phosphorylation Source: HGNC-UCL
Protein phosphorylation Source: GO_Central
Regulation of glucose metabolic process Source: GO_Central
Spermatogenesis Source: Ensembl
Valine catabolic process Source: Ensembl

Cellular Location

Mitochondrion matrix; Mitochondrion

Involvement in disease

Branched-chain ketoacid dehydrogenase kinase deficiency (BCKDKD): This suggests that it may be possible to treat patients with mutations in BCKDK with BCAA supplementation.
Disease descriptionA metabolic disorder characterized by autism, epilepsy, intellectual disability, and reduced branched-chain amino acids.

PTM

Autophosphorylated.

Nyhan, W. L., Al-Aqeel, A. I., & Barshop, B. A. (2020). Branched chain keto acid dehydrogenase kinase (BCKDK) deficiency. In Atlas of Inherited Metabolic Diseases (pp. 174-175). CRC Press.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Rabbit Anti-BCKDK Recombinant Antibody (CBYY-0300) (CAT#: CBMAB-0301-YY)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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