Recombinant Human Lysosomal acid lipase/LAL protein (Tagged) (ab152501)
Description
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Product name
Recombinant Human Lysosomal acid lipase/LAL protein (Tagged) -
Expression system
Wheat germ -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MKMRFLGLVVCLVLWPLHSEGSGGKLTALDPETNMNVSEIISYWGFPSEE YLVETEDGYILCLNRIPHGRKNHSDKGPKPVVFLQHGLLADSSNWVTNLA NSSLGFILADAGFDVWMGNSRGNTWSRKHKTLSVSQDEFWAFSYDEMAKY DLPASINFILNKTGQEQVYYVGHSQGTTIGFIAFSQIPELAKRIKMFFAL GPVASVAFCTSPMAKLGRLPDHLIKDLFGDKEFLPQSAFLKWLGTHVCTH VILKELCGNLCFLLCGFNERNLNMSRVDVYTTHSPAGTSVQNMLHWSQAV KFQKFQAFDWGSSAKNYFHYNQSYPPTYNVKDMLVPTAVWSGGHDWLADV YDVNILLTQITNLVFHESIPEWEHLDFIWGLDAPWRLYNKIINLMRKYQ -
Predicted molecular weight
70 kDa including tags -
Amino acids
1 to 399 -
Tags
GST tag N-Terminus
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Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab152501 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
ELISA
SDS-PAGE
Western blot
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Form
Liquid -
Additional notes
This product was previously labelled as Lysosomal acid lipase.
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Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.31% Glutathione, 0.79% Tris HCl
General Info
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Alternative names
- Acid cholesteryl ester hydrolase
- CESD
- cholesterol ester hydrolase
see all -
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. -
Involvement in 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.
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. -
Sequence similarities
Belongs to the AB hydrolase superfamily. Lipase family. -
Cellular localization
Lysosome. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human Lysosomal acid lipase/LAL protein (ab152501)12.5% SDS-PAGE analysis of ab152501 stained with Coomassie Blue.
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
References
ab152501 has not yet been referenced specifically in any publications.
