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Mouse Anti-LIPA Recombinant Antibody (CBYJL-1770) (CBMAB-L1681-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Lipase A Lysosomal Acid Type (LIPA). The antibody can be used for immunoassay techniques, such as WB.
See all LIPA antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYJL-1770
Antibody Isotype
IgG2b, κ
Application
WB

Basic Information

Immunogen
LIPA (AAH12287, 1 a.a. ~ 400 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 K.
Specificity
Human
Antibody Isotype
IgG2b, κ
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!]

Buffer
PBS, pH 7.4
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 1-400

Target

Full Name
LIPA
Introduction
LIPA 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. LIPA (Lipase A, Lysosomal Acid Type) is a Protein Coding gene. Diseases associated with LIPA include Lysosomal Acid Lipase Deficiency and Cholesterol Ester Storage Disease. Among its related pathways are Cholesterol and Sphingolipids transport / Distribution to the intracellular membrane compartments (normal and CF) and Metabolism. Gene Ontology (GO) annotations related to this gene include lipase activity and sterol esterase activity. An important paralog of this gene is LIPM.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.
Entrez Gene ID
UniProt ID
Alternative Names
LAL; CESD
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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