| 產品編號 | bs-18592R |
| 英文名稱 | Lysosomal acid lipase Rabbit pAb |
| 中文名稱 | 膽固醇酯水解酶抗體 |
| 別 名 | Acid Cholesteryl Ester Hydrolase; CESD; cholesterol ester hydrolase; cholesterol ester storage disease; Cholesteryl Esterase; Hydrolase deficiency; LAL; LAL deficiency cholesterol ester; LICH_HUMAN; LIPA; LIPA deficiency; Lipase A; lipase A, lysosomal acid, cholesterol esterase; lysosomal acid lipase; lysosomal acid lipase deficiency; Lysosomal acid lipase/cholesteryl ester hydrolase; Sterol esterase. |
| 研究領域 | 腫瘤 細胞生物 信號轉導 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應 | (predicted: Human,Mouse,Rat,Sheep,Cow,Horse) |
| 產品應用 | 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 | 43 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Lysosomal acid lipase: 101-200/399 |
| 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 |
| 產品介紹 |
This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jan 2014] Function: Crucial for the intracellular hydrolysis of cholesteryl esters and triglycerides that have been internalized via receptor-mediated endocytosis of lipoprotein particles. Important in mediating the effect of LDL (low density lipoprotein) uptake on suppression of hydroxymethylglutaryl-CoA reductase and activation of endogenous cellular cholesteryl ester formation. Subcellular Location: Lysosome. DISEASE: Defects in LIPA are the cause of Wolman disease (WOD) [MIM:278000]. WOD is a severe manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. WOD occurs in infancy and is nearly always fatal before the age of 1 year. Defects in LIPA are the cause of cholesteryl ester storage disease (CESD) [MIM:278000]. CESD is a mild manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. It is characterized by late-onset. Similarity: Belongs to the AB hydrolase superfamily. Lipase family. SWISS: P38571 Gene ID: 3988 Database links: Entrez Gene: 3988 Human Omim: 278000 Human SwissProt: P38571 Human Unigene: 643030 Human |
| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關于肽鏈的設計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
