Lysosomal Acid Lipase Deficiency Unmasked in Two Children With Nonalcoholic Fatty Liver Disease

PEDIATRICS ◽  
2016 ◽  
Vol 138 (4) ◽  
pp. e20160214-e20160214 ◽  
Author(s):  
R. W. Himes ◽  
S. E. Barlow ◽  
K. Bove ◽  
N. M. Quintanilla ◽  
R. Sheridan ◽  
...  
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Nonalcoholic Fatty Liver Disease ◽  
Fatty Liver Disease ◽  
Acid Lipase ◽  
Nonalcoholic Fatty Liver ◽  
Lysosomal Acid ◽  
Lysosomal Acid Lipase ◽  
Lysosomal Acid Lipase Deficiency

An overview of deregulated lipid metabolism in nonalcoholic fatty liver disease with special focus on lysosomal acid lipase

2020 ◽  
Vol 319 (4) ◽  
pp. G469-G480
Author(s):  
Simone Carotti ◽  
Katia Aquilano ◽  
Francesco Valentini ◽  
Sergio Ruggiero ◽  
Francesca Alletto ◽  
...  
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Nonalcoholic Fatty Liver Disease ◽  
Fatty Liver Disease ◽  
Fat Metabolism ◽  
Special Focus ◽  
Acid Lipase ◽  
Nonalcoholic Fatty Liver ◽  
Lysosomal Acid ◽  
Hepatic Fat

Obesity and type 2 diabetes are frequently complicated by excess fat accumulation in the liver, which is known as nonalcoholic fatty liver disease (NAFLD). In this context, liver steatosis develops as a result of the deregulation of pathways controlling de novo lipogenesis and fat catabolism. Recent evidences suggest the clinical relevance of a reduction in the activity of lysosomal acid lipase (LAL), which is a key enzyme for intracellular fat disposal, in patients with NAFLD. In this review, we provided a comprehensive overview of the critical steps in hepatic fat metabolism and alterations in these pathways in NAFLD, with a special focus on lipophagy and LAL activity. During NAFLD, hepatic fat metabolism is impaired at several levels, which is significantly contributed to by impaired lipophagy, in which reduced LAL activity may play an important role. For further research and intervention in NAFLD, targeting LAL activity may provide interesting perspectives.

scholarly journals Reduced Lysosomal Acid Lipase Activity in Adult Patients With Non-alcoholic Fatty Liver Disease

EBioMedicine ◽  
2015 ◽  
Vol 2 (7) ◽  
pp. 750-754 ◽  
Author(s):  
Francesco Baratta ◽  
Daniele Pastori ◽  
Maria Del Ben ◽  
Licia Polimeni ◽  
Giancarlo Labbadia ◽  
...  
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Lipase Activity ◽  
Fatty Liver Disease ◽  
Adult Patients ◽  
Acid Lipase ◽  
Alcoholic Fatty Liver ◽  
Lysosomal Acid ◽  
Lysosomal Acid Lipase ◽  
Alcoholic Fatty Liver Disease
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