|Tested species reactivity||Human, Mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||A synthetic peptide made to an internal region of the human Lysosomal acid lipase protein (within residues 150-300).|
|Purification||Antigen affinity chromatography|
|Storage buffer||PBS with 0.1% BSA, 50% glycerol|
|Contains||0.05% sodium azide|
|Storage Conditions||Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.|
|Tested Applications||Dilution *|
|Immunohistochemistry (Paraffin) (IHC (P))||1:100|
|Western Blot (WB)||1:10,000|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
The target sequence has 82% sequence homology with porcine and 73% sequence homology with rat and bovine.
Lipase A is a critical enzyme for metabolism, sequestration and degradation of cholesterol. When LIPA is not effective in liver and kidney cells, lipoprotein cholesterol accumulates and leads to atherosclerosis, inflammation, Type 2 diabetes, and chronic kidney disease. In Wolman disease, deficiency of LIPA causes buildup of cholesterol in spleen, liver, bone marrow, intestine, adrenal glands and lymph nodes, which eventually causes such toxicity in children affected that they rarely live past 1 year of age. Intracellularly, LIPA mediates the uptake of cholesterol by the endosomal/lysosomal compartment, and hydrolyzes cholesterol esterases.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.