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说明书
说明书
说明书
多克隆
Rabbit
说明书
HIBCH
Hib乙酰辅酶A水解酶抗体
Unconjugated
说明书
说明书
KLH conjugated synthetic peptide derived from human HIBCH
LgG
液体
说明书
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
1mg/ml
100ul
产品名称:Hib乙酰辅酶A水解酶抗体 英文名称:HIBCH 英文别名:3 hydroxyisobutyryl Coenzyme A hydrolase; 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE; HIB CoA hydrolase; HIB-CoA hydrolase; HIBCH; HIBCH_HUMAN; HIBCoA hydrolase; HIBYL CoA H; HIBYL CoAH; HIBYL-CoA-H; HIBYLCoA H; HIBYLCoAH; mitochondrial; HIBCH_HUMAN. 交叉反应: Human 标记: Unconjugated 抗体来源: Rabbit 抗体类型: Polyclonal 免疫原: KLH conjugated synthetic peptide derived from human HIBCH 蛋白细胞定位: 细胞浆 纯化方法: affinity purified by Protein A 亚型: IgG 性状: Liquid 浓度: 1mg/ml 储存液: 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. 保存条件: Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 背景资料: HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localizing to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyzes HIBYL-CoA, a saline catabolite, and β-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of β-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterized by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia. |
产品名称 | 浓度 | 型号 |
Hib乙酰辅酶A水解酶抗体 | 1mg/ml | EY-01K6259 |
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