| 產(chǎn)品編號 | bs-5067R |
| 英文名稱 | Rabbit Anti-HMGCL antibody |
| 中文名稱 | 三羥基三甲基輔酶A裂解酶抗體 |
| 別 名 | 3 hydroxy 3 methylglutaryl CoA lyase; 3 hydroxy 3 methylglutaryl Coenzyme A lyase; 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria); 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase; 3-hydroxy-3-methylglutarate-CoA lyase; HL; HMG CoA lyase; HMG CoA Lyase Deficiency; HMG-CoA lyase; HMGCL; HMGCL_HUMAN; Hydroxymethylglutaricaciduria; Hydroxymethylglutaryl CoA lyase; Hydroxymethylglutaryl CoA lyase mitochondrial; Hydroxymethylglutaryl-CoA lyase; mitochondrial; MS725; OTTHUMP00000044830. |
| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 免疫學(xué) 信號轉(zhuǎn)導(dǎo) 脂蛋白 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse (predicted: Human,Rat,Rabbit,Chicken,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=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 | 32kDa |
| 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 HMGCL: 41-140/325 |
| 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 | 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 |
Hydroxymethylglutaryl-CoA lyase (HMGCL) is found in fibroblasts, liver and lymphoblasts. It has a role in ketogenesis and leucine catabolism. Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (hydroxymethylglutaricaciduria), an autosomal recessive disease which can lead to hypoglycemia and coma. Function: Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Subunit: Homodimer; disulfide-linked. Can also form homotetramers. Subcellular Location: Mitochondrion matrix. Tissue Specificity: Fibroblasts, liver and lymphoblasts. DISEASE: Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases. Similarity: Belongs to the HMG-CoA lyase family. SWISS: P35914 Gene ID: 3155 Database links: Entrez Gene: 3155 Human Entrez Gene: 15356 Mouse Omim: 246450 Human Omim: 613898 Human SwissProt: P35914 Human SwissProt: P38060 Mouse Unigene: 533444 Human Unigene: 482102 Mouse Unigene: 12297 Rat |
| 產(chǎn)品圖片 |
Sample:
Liver (Mouse) Lysate at 40 ug
Primary: Anti- HMGCL (bs-5067R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 32 kD
Observed band size: 30 kD
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