Recombinant Human LIPA Protein, GMP Grade


Cat# Product Name Availability Size Price Qty
LIPA-080THP Recombinant Human LIPA Protein, GMP Grade 10ug $998.00
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Cat#:  LIPA-080THP
Common Name:  LIPA
Product Name:  Recombinant Human LIPA Protein, GMP Grade
Product Overview:  Recombinant Human LIPA Protein without tag was produced in an animal component free process under cGMP guidelines.
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 have been found for this gene.
Species:  Human
Bio-activity:  2 mg/mL
Molecular Mass:  55000.0 Da
AA Sequence:  SGGKLTAVDPETNMNVSEIISYWGFPSEEYLVETEDGYILCLNRIPHGRKNHSDKGPKPVVFLQHGLLADSSNWVTNLANSSLGFILADAGFDVWMGNSRGNTWSRKHKTLSVSQDEFWAFSYDEMAKYDLPASINFILNKTGQEQVYYVGHSQGTTIGFIAFSQIPELAKRIKMFFALGPVASVAFCTSPMAKLGRLPDHLIKDLFGDKEFLPQSAFLKWLGTHVCTHVILKELCGNLCFLLCGFNERNLNMSRVDVYTTHSPAGTSVQNMLHWSQAVKFQKFQAFDWGSSAKNYFHYNQSYPPTYNVKDMLVPTAVWSGGHDWLADVYDVNILLTQITNLVFHESIPEWEHLDFIWGLDAPWRLYNKIINLMRKYQ
Endotoxin:  <0.001 EU/μg of the peptide by the LAL method
Purity:  > 99 % by SDS-PAGE and HPLC analysis
Applications:  The product is a hydrolytic lysosomal cholesteryl ester and triacylglycerol-specific enzyme indicated for the treatment of patients with a diagnosis of Lysosomal Acid Lipase (LAL) deficiency.
Usage:  Lysosomal Acid Lipase (LAL) deficiency
Gene Name:  LIPA lipase A, lysosomal acid, cholesterol esterase [ Homo sapiens (human) ]
Official Symbol:  LIPA
Synonyms:  LIPA; lipase A, lysosomal acid, cholesterol esterase; lysosomal acid lipase/cholesteryl ester hydrolase; CESD; LAL; Wolman disease; sterol esterase; cholesteryl esterase; lysosomal acid lipase; cholesterol ester hydrolase; acid cholesteryl ester hydrolase;
GeneID:  3988
mRNA Refseq:  NM_000235
Protein Refseq:  NP_000226
MIM:  613497
UniProt ID:  P38571
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