Lysosomal Acid Lipase: From Cellular Lipid Handler to Immunometabolic Target
نویسندگان
چکیده
منابع مشابه
Lysosomal acid lipase in cancer
Lysosomal Acid Lipase Regulates Myeloid-derived Suppressor Cells to Control Cancer Cell Proliferation and Metastasis. Inflammation critically contributes to cancer growth and metastasis, in which myeloid-derived suppressor cells (MDSCs) are an important participant. MDSCs are known to suppress immune surveillance to promote tumorigenesis [1]. Lysosomal acid lipase (LAL), a critical enzyme in co...
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We report the biological characterization of an animal model of a genetic lipid storage disease analogous to human Wolman's disease. Affected rats accumulated cholesteryl esters (13.3-fold), free cholesterol (2.8-fold), and triglycerides (5.4-fold) in the liver, as well as cholesteryl esters (2.5-fold) and free cholesterol (1.33-fold) in the spleen. Triglycerides did not accumulate, and the lev...
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OBJECTIVE The aim of this study was to characterize key clinical manifestations of lysosomal acid lipase deficiency (LAL D) in children and adults. METHODS Investigators reviewed medical records of LAL D patients ages ≥5 years, extracted historical data, and obtained prospective laboratory and imaging data on living patients to develop a longitudinal dataset. RESULTS A total of 49 patients ...
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INTRODUCTION Lysosomal acid lipase deficiency (LAL-D) causes progressive cholesteryl ester and triglyceride accumulation in the lysosomes of hepatocytes and monocyte-macrophage system cells, resulting in a systemic disease with various manifestations that may go unnoticed. It is indispensable to recognize the deficiency, which can present in patients at any age, so that specific treatment can b...
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ژورنال
عنوان ژورنال: Trends in Pharmacological Sciences
سال: 2019
ISSN: 0165-6147
DOI: 10.1016/j.tips.2018.12.006