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

Details for Product anti-LIPA Antibody No. ABIN969484, Supplier: Log in to see
Antigen
  • cesd
  • lal
  • LIPA
  • CESD
  • LAL
  • Chole
  • Chole2
  • Lip1
  • AA960673
  • Lal
  • Lip-1
  • lipase A, lysosomal acid, cholesterol esterase
  • lysosomal acid lipase A
  • LIPA
  • lipa
  • Lipa
Reactivity
Human
84
12
9
2
2
1
1
1
1
Host
Mouse
51
35
3
Clonality (Clone)
Monoclonal ()
Conjugate
This Lipase A antibody is un-conjugated
7
7
7
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
Application
ELISA, Western Blotting (WB)
73
51
16
5
4
4
3
2
2
1
Options
Supplier
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Immunogen Purified recombinant fragment of LAL expressed in E. Coli.
Clone 9G7F12
Isotype IgG2a
Purification Purified
Alternative Name LAL (LIPA Antibody Abstract)
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.
Gene ID 3988
HGNC 3988
Application Notes ELISA: 1:10000, WB: 1:500 - 1:2000
Restrictions For Research Use only
Format Liquid
Buffer PBS containing 0.03 % 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 4°C, -20°C for long term storage
Supplier Images
Western Blotting (WB) image for anti-Lipase A antibody (Lipase A, Lysosomal Acid, Cholesterol Esterase) (ABIN969484) Figure 1: Western blot analysis using LAL mouse mAb against LAL recombinant protein.
Product cited in: Drebber, Andersen, Kasper, Lohse, Stolte, Dienes: "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).

Boldrini, Devito, Biselli, Filocamo, Bosman: "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).