Mouse anti-Human Lysosomal Acid Lipase Polyclonal Antibody | anti-LIPA antibody
Lysosomal Acid Lipase (LAL, Acid Cholesteryl Ester Hydrolase, Cholesteryl Esterase, Lipase A, LIPA, Sterol Esterase)
Purified.
Purified.
NCBI and Uniprot Product Information
NCBI Description
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 encoding the same protein have been found for this gene. [provided by RefSeq]
Uniprot Description
LIPA: 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. Defects in LIPA are the cause of Wolman disease (WOD). 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). 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. Belongs to the AB hydrolase superfamily. Lipase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Lipid Metabolism - steroid biosynthesis; EC 3.1.1.13; Hydrolase
Chromosomal Location of Human Ortholog: 10q23.2-q23.3
Cellular Component: lysosome
Molecular Function: sterol esterase activity; lipase activity
Biological Process: cell proliferation; homeostasis of number of cells within a tissue; tissue remodeling; cell morphogenesis; cytokine production; inflammatory response; lipid catabolic process; lung development
Disease: Lysosomal Acid Lipase Deficiency
Research Articles on LIPA
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Product Notes
The LIPA lipa (Catalog #AAA626135) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Lysosomal Acid Lipase (LAL, Acid Cholesteryl Ester Hydrolase, Cholesteryl Esterase, Lipase A, LIPA, Sterol Esterase) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Lysosomal Acid Lipase can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Suitable for use in Western Blot. Researchers should empirically determine the suitability of the LIPA lipa for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Lysosomal Acid Lipase, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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