Reduced lysosomal acid lipase activity: A new marker of liver disease severity across the clinical continuum of non-alcoholic fatty liver disease?
Reduced lysosomal acid lipase activity: A new marker of liver disease severity across the clinical continuum of non-alcoholic fatty liver disease?作者机构：Department of Internal Medicine and Medical SpecialtiesSapienza-University of RomeRome 00155Italy Hepatogastroenterology and Nutrition Unit-Pediatric DepartmentBambino GesùChildren’s HospitalRome 00156Italy Department of Public Health and Infectious DiseaseSapienza-University of RomeRome 00161Italy
出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)
年 卷 期：2019年第25卷第30期
页 面：4172-4180页
核心收录：
学科分类：10[医学]
主 题：Non-alcoholic fatty liver disease Non-alcoholic steatohepatitis Lysosomal acid lipase Cirrhosis Wolman disease Cholesterol ester storage disease
摘 要：Lysosomal acid lipase(LAL)plays a key role in intracellular lipid *** LAL activity promotes increased multi-organ lysosomal cholesterol ester storage,as observed in two recessive autosomal genetic diseases,Wolman disease and Cholesterol ester storage *** liver steatosis and accelerated liver fibrosis are common features in patients with genetic LAL *** contrast,few reliable data are available on the modulation of LAL activity in vivo and on the epigenetic and metabolic factors capable of regulating its activity in subjects without homozygous mutations of the Lipase A *** the last few years,a less severe and non-genetic reduction of LAL activity was reported in children and adults with non-alcoholic fatty liver disease(NAFLD),suggesting a possible role of LAL reduction in the pathogenesis and progression of the *** with NAFLD show a significant,progressive reduction of LAL activity from simple steatosis to non-alcoholic steatohepatitis and cryptogenic *** cirrhosis of different etiologies,those with cryptogenic cirrhosis show the most significant reductions of LAL *** findings suggest that the modulation of LAL activity may become a possible new therapeutic target for patients with more advanced forms of ***,the measurement of LAL activity may represent a possible new marker of disease severity in this clinical setting.
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