| 產(chǎn)品編號 | bs-15484R |
| 英文名稱 | Rabbit Anti-HIBCH antibody |
| 中文名稱 | Hib乙酰輔酶A水解酶抗體 |
| 別 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. |
| 研究領(lǐng)域 | 信號轉(zhuǎn)導(dǎo) 新陳代謝 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 39kDa |
| 細(xì)胞定位 | 細(xì)胞漿 線粒體 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HIBCH : 251-350/386 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. Function: 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. Subcellular Location: Mitochondrion (By similarity). Tissue Specificity: Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. DISEASE: HIBCH deficiency (HIBCHD) [MIM:250620]: The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the enoyl-CoA hydratase/isomerase family. SWISS: Q6NVY1 Gene ID: 26275 Database links: Entrez Gene: 26275 Human Omim: 610690 Human SwissProt: Q6NVY1 Human Unigene: 656685 Human |
