For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Small volumes of anti-LIPA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC)

Western blot analysis of extracts of various cell lines, using LIPA antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 90s.

399

lipase A, lysosomal acid, cholesterol esterase

lysosomal acid lipase/cholesteryl ester hydrolase; sterol esterase; cholesteryl esterase; cholesterol ester hydrolase; acid cholesteryl ester hydrolase

Lysosomal acid lipase/cholesteryl ester hydrolase

Acid cholesteryl ester hydrolase; LAL??[Similar Products]

LICH_HUMAN

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]

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: EC 3.1.1.13; Lipid Metabolism - steroid biosynthesis; 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

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