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Anti-BCKD Antibody

品牌：Antibodies

货号：

规格：50µl

货期：

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Anti-BCKD Antibody

商品详情参考文献相关资料

Name:	Anti-BCKD Antibody See all BCKD primary antibodies
Description:	Rabbit polyclonal antibody to BCKD
Specificity:	The antibody detects endogenous levels of total BCKD protein.
Applications:	WB, IF
Reactivity:	Human, Mouse, Rat
Immunogen:	Synthesized peptide derived from Internal of human BCKD.
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Purification:	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:	1.0mg / ml
Formulation:	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage:	Store at -20˚C

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.
Tissue Specificity:	Ubiquitous.
Involvement in Disease:	Branched-chain ketoacid dehydrogenase kinase deficiency: A metabolic disorder characterized by autism, epilepsy, intellectual disability, and reduced branched-chain amino acids.
Sequence Similarities:	Belongs to the PDK/BCKDK protein kinase family.
Post-Translational Modification:	Autophosphorylated.
Cellular Location:	Mitochondrion matrix. Mitochondrion.