ITM0410
ITM0410
ITM0410
from
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Price in reward points: 556
- Catalog: ITM0410
- Gene/Protein: LIPA
- Product Description: Immunotag™ LAL Monoclonal Antibody
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Description
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein LAL Clonality Monoclonal Storage/Stability -20°C/1 year Application WB,ELISA Recommended Dilution Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. Concentration 1 mg/ml Reactive Species Human Host Species Mouse Immunogen Purified recombinant fragment of LAL expressed in E. Coli. Specificity LAL Monoclonal Antibody detects endogenous levels of LAL protein. Purification Affinity purification Form Purified antibody in PBS containing 0.03% sodium azide. Gene Name LIPA Accession No. P38571 Q9Z0M5 Alternate Names LIPA; Lysosomal acid lipase/cholesteryl ester hydrolase; Acid cholesteryl ester hydrolase; LAL; Cholesteryl esterase; Lipase A; Sterol esterase Description lipase A, lysosomal acid type(LIPA) Homo sapiens 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], Cell Pathway/ Category Steroid biosynthesis,Lysosome, Protein Expression Brain,Liver,Placenta,Umbilical cord blood, Subcellular Localization lysosome,extracellular exosome, Protein Function catalytic activity:A steryl ester + H(2)O = a sterol + a fatty acid.,disease: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.,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.,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.,similarity:Belongs to the AB hydrolase superfamily. Lipase family., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | LAL |
| Clonality | Monoclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human |
| Host Species | Mouse |
| Immunogen | Purified recombinant fragment of LAL expressed in E. Coli. |
| Specificity | LAL Monoclonal Antibody detects endogenous levels of LAL protein. |
| Purification | Affinity purification |
| Form | Purified antibody in PBS containing 0.03% sodium azide. |
| Gene Name | LIPA |
| Accession No. | P38571 Q9Z0M5 |
| Alternate Names | LIPA; Lysosomal acid lipase/cholesteryl ester hydrolase; Acid cholesteryl ester hydrolase; LAL; Cholesteryl esterase; Lipase A; Sterol esterase |
| Description | lipase A, lysosomal acid type(LIPA) Homo sapiens 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], |
| Cell Pathway/ Category | Steroid biosynthesis,Lysosome, |
| Protein Expression | Brain,Liver,Placenta,Umbilical cord blood, |
| Subcellular Localization | lysosome,extracellular exosome, |
| Protein Function | catalytic activity:A steryl ester + H(2)O = a sterol + a fatty acid.,disease: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.,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.,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.,similarity:Belongs to the AB hydrolase superfamily. Lipase family., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |