BCKDK Antibody (N-term) Blocking Peptide
€363.00
In stock
SKU
AC-BP7090a
Background:
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 ubiquitiously 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.
Other Names:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
Target/Specificity:
The synthetic peptide sequence used to generate the antibody AP7090a was selected from the N-term region of human BCKDK. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Gene Name: BCKDK {ECO:0000303|PubMed:29779826, ECO:0000312|HGNC:HGNC:16902}
Gene ID: 10295
Primary Accession: O14874
Format: Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
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 ubiquitiously 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.
Other Names:
[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
Target/Specificity:
The synthetic peptide sequence used to generate the antibody AP7090a was selected from the N-term region of human BCKDK. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
Gene Name: BCKDK {ECO:0000303|PubMed:29779826, ECO:0000312|HGNC:HGNC:16902}
Gene ID: 10295
Primary Accession: O14874
Format: Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
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