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- Bioss
- bs-15484P
- 2025年10月16日
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500ug
| 产品编号 | bs-15484P |
| 英文名称 | HIBCH Antibody Blocking Peptide |
| 中文名称 | Hib乙酰辅酶A水解酶封闭多肽 |
| 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. |
| 纯化方法 | HPLC |
| 研究领域 | Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism Signal Transduction > Metabolism > Amino Acids |
| 亚细胞定位 | Mitochondrion (By similarity). |
| 组织特异性 | Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. |
| 相似性 | Belongs to the enoyl-CoA hydratase/isomerase family. |
| 功能 | Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 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. |
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HIBCH Antibody Blocking Peptide(bs-15484P)-500ug
￥880
