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Lipase A, Lysosomal Acid, Cholesterol Esterase (LIPA) antibody

Details for Product No. ABIN659253
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Antigen
Synonyms LAL, CESD, Lip1, Chole, Chole2, AA960673, Lal, Lip-1, cesd, lal, LIPA
Reactivity
Human
(27), (5), (2)
Host
Mouse
(14), (13)
Clonality
Monoclonal
Application
Western Blotting (WB)
(25), (16), (3), (3), (2), (2), (2), (2), (1), (1)
Pubmed 2 references available
Quantity 0.1 mg
Options
Shipping to United States (Change)
Availability Will be delivered in 2 to 3 Business Days
Catalog No. ABIN659253
330.00 $
Plus shipping costs $45.00

Order hotline:

  • +1 404 474 4654
  • +1 888 205 9894 (TF)
Immunogen Purified recombinant fragment of LAL expressed in E. Coli.
Isotype IgG2a
Specificity Purified recombinant fragment of LAL expressed in E. Coli.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Alternative Name LAL
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 45419 DA
Gene ID 12406
UniProt P38571, NP_001121077.1
Research Area Metabolism, Cell Structure
Application Notes Western Bloting: = 1:500 - 2000.
Comment

Background: Lysosomal acid lipase (LAL), with 378-amino acid protein( 43-54 kDa), functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides which are taken up by receptor-mediated endocytosis. An inherited deficiency or low activity of human lysosomal acid lipase results in the intralysosomal storage of the respective lipid substrates. So it is also responsible for the rare conditions of Wolman disease and cholesteryl ester storage disease (CESD). As the enzyme is synthesized by all nucleated cells, lipid-laden cells are found in all organs, particularly in liver, spleen, the adrenal and the hemopoietic system, and in the intestine as well as in the lymph nodes, lungs, testes, and ovaries.

Restrictions For Research Use only
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Background publications Boldrini, Devito, Biselli et al.: "Wolman disease and cholesteryl ester storage disease diagnosed by histological and ultrastructural examination of intestinal and liver biopsy." in: Pathology, research and practice, Vol. 200, Issue 3, pp. 231-40, 2004 (PubMed).

Drebber, Andersen, Kasper et al.: "Severe chronic diarrhea and weight loss in cholesteryl ester storage disease: a case report." in: World journal of gastroenterology : WJG, Vol. 11, Issue 15, pp. 2364-6, 2005 (PubMed).

Request Want additional data for this product?

The Independent Validation Initiative strives to provide you with high quality data. Find out more

Catalog No. ABIN659253
330.00 $
Plus shipping costs $45.00

Order hotline:

  • +1 404 474 4654
  • +1 888 205 9894 (TF)
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