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产品信息
BCKD-101AP
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Synthetic peptide taken within amino acid region 170-200 on human BCKD protein.
偶联物
Unconjugated
形式
Liquid
浓度
0.5-1.5 mg/mL
纯化类型
Affinity chromatography
保存液
proprietary buffer, pH 7.4-7.8, with 30% glycerol, 0.5% BSA
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Ambient (domestic); Wet ice (international)
靶标信息
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.
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生物信息学
蛋白别名: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKD-kinase; BCKDH kinase; BCKDHKIN; branched chain alpha-ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
基因别名: AI327402; BCKDK; BCKDKD; BDK
UniProt ID: (Human) O14874, (Rat) Q00972, (Mouse) O55028
Entrez Gene ID: (Human) 10295, (Rat) 29603, (Mouse) 12041
protein serine/threonine kinase activity
protein binding
ATP binding
kinase activity
[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity
protein kinase activity
nucleotide binding
transferase activity
transferase activity, transferring phosphorus-containing groups
non-receptor serine/threonine protein kinase
