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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against LIPA. It has beenselected for its ability to recognize LIPA in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.5-2ug/mL;1:400-1600
Immunohistochemistry: 5-20ug/mL;1:40-160
Immunocytochemistry: 5-20ug/mL;1:40-160
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin-embedded Liver tissue)

45,325 Da

lysosomal acid lipase A

lysosomal acid lipase/cholesteryl ester hydrolase

Lysosomal acid lipase/cholesteryl ester hydrolase

Lip1; Acid cholesteryl ester hydrolase; LAL??[Similar Products]

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; Hydrolase; Lipid Metabolism - steroid biosynthesis

Chromosomal Location of Human Ortholog: 19|19 C1

Cellular Component: fibrillar center; intracellular membrane-bound organelle; lysosome

Molecular Function: hydrolase activity; hydrolase activity, acting on ester bonds; lipase activity; sterol esterase activity

Biological Process: cell morphogenesis; cell proliferation; cytokine production; fatty acid metabolic process; homeostasis of number of cells within a tissue; inflammatory response; lipid catabolic process; lipid metabolic process; lung development; sterol metabolic process; tissue remodeling

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