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Mouse Anti-LIPA Recombinant Antibody (9G7F12,7G6D7) (CBMAB-AO689LY)

The product is antibody recognizes LIPA. The antibody 9G7F12,7G6D7 immunoassay techniques such as: ELISA, WB.
See all LIPA antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
9G7F12,7G6D7
Antibody Isotype
IgG2a
Application
ELISA, WB

Basic Information

Immunogen
Purified recombinant fragment of LAL expressed in E. coli.
Specificity
Human
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Preservative
0.03% sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
LIPA
Entrez Gene ID
3988
UniProt ID
P38571
Alternative Names
Lysosomal acid lipase/cholesteryl ester hydrolase; Acid cholesteryl ester hydrolase; LAL; Cholesteryl esterase; Lipase A; Sterol esterase
Function
Catalyzes the deacylation of triacylglyceryl and cholesteryl ester core lipids of endocytosed low density lipoproteins to generate free fatty acids and cholesterol.
Biological Process
Cell morphogenesisIEA:Ensembl
Cell population proliferationIEA:Ensembl
Homeostasis of number of cells within a tissueIEA:Ensembl
Inflammatory responseIEA:Ensembl
Lipid catabolic processIEA:UniProtKB-KW
Low-density lipoprotein particle clearanceTAS:Reactome
Lung developmentIEA:Ensembl
Sterol metabolic processManual Assertion Based On ExperimentIBA:GO_Central
Tissue remodelingIEA:Ensembl
Cellular Location
Lysosome
Involvement in disease
Wolman disease (WOD):
A severe manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. WD occurs in infancy and is nearly always fatal before the age of 1 year.
Cholesteryl ester storage disease (CESD):
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.
PTM
Glycosylation is not essential for catalytic activity.

Korbelius, M., Kuentzel, K. B., Bradić, I., Vujić, N., & Kratky, D. (2023). Recent insights into lysosomal acid lipase deficiency. Trends in molecular medicine.

Gamblin, C., Rouault, C., Lacombe, A., Langa-Vives, F., Farabos, D., Lamaziere, A., ... & Dugail, I. (2021). Lysosomal acid lipase drives adipocyte cholesterol homeostasis and modulates lipid storage in obesity, independent of autophagy. Diabetes, 70(1), 76-90.

Pastores, G. M., & Hughes, D. A. (2020). Lysosomal acid lipase deficiency: therapeutic options. Drug design, development and therapy, 591-601.

Carotti, S., Aquilano, K., Valentini, F., Ruggiero, S., Alletto, F., Morini, S., ... & Vespasiani-Gentilucci, U. (2020). An overview of deregulated lipid metabolism in nonalcoholic fatty liver disease with special focus on lysosomal acid lipase. American Journal of Physiology-Gastrointestinal and Liver Physiology, 319(4), G469-G480.

Gomaraschi, M., Bonacina, F., & Norata, G. D. (2019). Lysosomal acid lipase: from cellular lipid handler to immunometabolic target. Trends in pharmacological sciences, 40(2), 104-115.

Li, F., & Zhang, H. (2019). Lysosomal acid lipase in lipid metabolism and beyond. Arteriosclerosis, thrombosis, and vascular biology, 39(5), 850-856.

Evans, T. D., Zhang, X., Clark, R. E., Alisio, A., Song, E., Zhang, H., ... & Razani, B. (2019). Functional characterization of lipa (lysosomal acid lipase) variants associated with coronary artery disease. Arteriosclerosis, thrombosis, and vascular biology, 39(12), 2480-2491.

Strebinger, G., Müller, E., Feldman, A., & Aigner, E. (2019). Lysosomal acid lipase deficiency–early diagnosis is the key. Hepatic medicine: evidence and research, 79-88.

Del Angel, G., Hutchinson, A. T., Jain, N. K., Forbes, C. D., & Reynders, J. (2019). Large‐scale functional LIPA variant characterization to improve birth prevalence estimates of lysosomal acid lipase deficiency. Human mutation, 40(11), 2007-2020.

Bychkov, I. O., Kamenets, E. A., Filatova, A. Y., Skoblov, M. Y., Mikhaylova, S. V., Strokova, T. V., ... & Zakharova, E. Y. (2019). The novel synonymous variant in LIPA gene affects splicing and causes lysosomal acid lipase deficiency. Molecular Genetics and Metabolism, 127(3), 212-215.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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