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Fabgennix
BCKDH Polyclonal Antibody, FITC
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Product Details
BCKD-FITC
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Synthetic peptide taken within amino acid region 170-200 on human BCKD protein.
Conjugate
Excitation/Emission Max
498/517 nm
View spectra
Form
Liquid
Concentration
0.5-1.5 mg/mL
Purification
Affinity chromatography
Storage buffer
proprietary buffer, pH 7.4-7.8, with 30% glycerol, 0.5% BSA
Contains
0.02% sodium azide
Storage conditions
-20°C, store in dark
Shipping conditions
Ambient (domestic); Wet ice (international)
Target Information
The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKD kinase; BCKD-kinase; BCKDH kinase; BCKDHKIN; BCKDK; branched chain alpha-ketoacid dehydrogenase kinase; branched chain ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4; unnamed protein product
Gene Aliases: AI327402; BCKDK; BCKDKD; BDK
UniProt ID: (Human) O14874, (Rat) Q00972, (Mouse) O55028
Entrez Gene ID: (Human) 10295, (Rat) 29603, (Mouse) 12041
nucleotide binding
protein kinase activity
protein serine/threonine kinase activity
protein serine/threonine phosphatase activity
pyruvate dehydrogenase (acetyl-transferring) kinase activity
protein binding
ATP binding
kinase activity
transferase activity
transferase activity, transferring phosphorus-containing groups
[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity
protein serine kinase activity
catalytic activity, acting on a protein
non-receptor serine/threonine protein kinase
isoleucine catabolic process
leucine catabolic process
valine catabolic process
spermatogenesis
lipid biosynthetic process
cellular amino acid catabolic process
branched-chain amino acid catabolic process
regulation of acetyl-CoA biosynthetic process from pyruvate
regulation of glucose metabolic process
protein phosphorylation
phosphorylation
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Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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