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Mouse Anti-BCKDK Recombinant Antibody (EG366) (CBMAB-EN403-LY)

The product is antibody recognizes BCKDK . The antibody EG366 immunoassay techniques such as: WB: 1:500~1:1000 IF: 1:100~1:500 ELISA: 1:20000.
See all BCKDK antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
EG366
Antibody Isotype
IgG1
Application
WB, IHC, IF

Basic Information

Immunogen
Full length human recombinant protein of human BCKDK produced in HEK293T cell.
Host Species
Mouse
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG1
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:2,000
IF(ICC)1:100
IHC1:50

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

Format
Liquid
Buffer
PBS, pH 7.3, 1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
Batch dependent
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Branched Chain Ketoacid Dehydrogenase Kinase
Introduction
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. [provided by RefSeq, Dec 2012]
Entrez Gene ID
Human10295
Mouse12041
Rat29603
UniProt ID
HumanO14874
MouseO55028
RatQ00972
Alternative Names
Branched Chain Ketoacid Dehydrogenase Kinase; BCKD-Kinase; EC 2.7.11.4; BCKDHKIN; [3-Methyl-2-Oxobutanoate Dehydrogenase [Lipoamide]] Kinase, Mitochondrial; 3-Methyl-2-Oxobutanoate Dehydrogenase [Lipoamide] Kinase, Mitochondrial;
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.
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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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