Mouse Anti-BCKDK Recombinant Antibody (CBYY-0299) (CBMAB-0300-YY)

Name: Mouse Anti-BCKDK Recombinant Antibody (CBYY-0299)
SKU: CBMAB-0300-YY
Rating: 5 (1 reviews)

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Published Data

Mouse Anti-BCKDK Recombinant Antibody (CBYY-0299) (CBMAB-0300-YY) in Immunoblot

Representative immunoblot data of E1α expression in liver and heart tissues from the Control and the BCKDHA-cKO mice. e Real-time PCR analysis of BCKDHA normalized to GAPDH in different tissues as indicated. n = 3 each group, *P < 0.05. ...View More

Yu, J. Y., Cao, N., Rau, C. D., Lee, R. P., Yang, J., Flach, R. J. R., ... & Gao, C. (2023). Cell-autonomous effect of cardiomyocyte branched-chain amino acid catabolism in heart failure in mice. Acta Pharmacologica Sinica, 1-11.

Mouse Anti-BCKDK Recombinant Antibody (CBYY-0299) (CBMAB-0300-YY) in IF

Immunofluorescence analysis of BCKDK in A549 cells was performed by fluorescence microscope. ...View More

Xue, M., Xiao, J., Jiang, W., Wang, Y., Zuo, D., An, H., & Ren, L. (2023). Loss of BCAA catabolism enhances Rab1A-mTORC1 signaling activity and promotes tumor proliferation in NSCLC. Translational Oncology, 34, 101696.

Mouse Anti-BCKDK Recombinant Antibody (CBYY-0299) (CBMAB-0300-YY) in WB

The BCKDK protein level of A549 BCKDK KD and BCKDK OV stable cell lines was measured using western blotting (n = 3 for each group). ...View More

Mouse Anti-BCKDK Recombinant Antibody (CBYY-0299) (CBMAB-0300-YY) in IHC

IHC staining of BCKDK in NSCLC. Representative images are shown. (200 ×, scale bar = 40 µm). ...View More

Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

CBYY-0299

Application

WB, IP, IF, ELISA

Immunogen

Amino acids 1-280 mapping at the N-terminus of BCKDK of human origin.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG1, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:100-1:1,000
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)	1:50-1:500
ELISA	1:100-1:1,000

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

Format

Liquid

Buffer

PBS, 0.1% gelatin

Preservative

< 0.1% sodium azide

Concentration

0.2 mg/ml

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

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 isoforMouse have been found for this gene. [provided by RefSeq, Dec 2012]

Entrez Gene ID

10295

UniProt ID

O14874

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.

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)
Proteogenomics
Other Protocols

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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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