Anti-BCKDK Antibody

$99.00$319.00

Reactivity: Human
Applications: ELISA,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-BCKDK Antibody (CSB-PA002607ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Branched-chain alpha-ketoacid dehydrogenase kinase Antibody,BCKD-kinase Antibody, BCKDHKIN Antibody,BCKDK Antibody

More alternative names for the antibody
[3 methyl 2 oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial antibody|[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase antibody|BCKD kinase antibody|BCKD-kinase antibody|BCKD_HUMAN antibody|BCKDHKIN antibody|Bckdk antibody|BCKDKD antibody|BDK antibody|Branched chain alpha keto acid dehydrogenase kinase antibody|Branched chain ketoacid dehydrogenase kinase antibody|Branched-chain alpha-ketoacid dehydrogenase kinase antibody|mitochondrial antibody
Anti-BCKDH kinase antibody (ab151297)
Close sc-374425|sc-374424|sc-163735|

Recommended applications: ELISA, IHC

Recommended dilution: Recommended dilution:IHC:1:20-1:200

Recommended protocols: check protocols

Name

Anti-BCKDK Antibody

Catalogue No.

CSB-PA002607ESR1HU

Reactivity

Human

Immunogen

Recombinant human [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial GeneBCKDK protein (153-412AA)

Host

Rabbit

Applications

ELISA, IHC

Recommended dilution

Recommended dilution:IHC:1:20-1:200

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

46 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:505005( Cow), Entrez Gene:10295( Human), Entrez Gene:12041( Mouse), Entrez Gene:29603( Rat), Omim:614901( Human), SwissProt:Q2KJG8( Cow), SwissProt:O14874( Human), SwissProt:O55028( Mouse), SwissProt:Q00972( Rat), Unigene:513520( Human), Unigene:425495( Mouse), Unigene:31976( Rat)

Protein 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. .

Protein tissue specificity

Ubiquitous.

Involvement in disease

Branched-chain ketoacid dehydrogenase kinase deficiency (BCKDKD) [MIM:614923]: A metabolic disorder characterized by autism, epilepsy, intellectual disability, and reduced branched-chain amino acids. . Note=The disease is caused by mutations affecting the gene represented in this entry. A diet enriched in branched amino acids (BCAAs) allows to normalize plasma BCAA levels. This suggests that it may be possible to treat patients with mutations in BCKDK with BCAA supplementation.

Protein sequence and domain

Belongs to the PDK/BCKDK protein kinase family.

Protein post-translational modifications

Autophosphorylated. .

Protein cellular localization

Mitochondrion matrix. Mitochondrion .

Research area

All research areas>Synthesis and Degradation>BCKDK
(View all antibody categories related to Synthesis and Degradation)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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