A Relative Deficiency of Lysosomal Acid Lypase Activity Characterizes Non-Alcoholic Fatty Liver Disease

• Published in: International journal of molecular sciences Vol. 18; no. 6; p. 1134
• Main Authors: Tovoli, Francesco, Napoli, Lucia, Negrini, Giulia, D'Addato, Sergio, Tozzi, Giulia, D'Amico, Jessica, Piscaglia, Fabio, Bolondi, Luigi
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 25.05.2017; MDPI
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Biomarkers; Blood; Blood platelets; Cholesterol; Cirrhosis; Diabetes; Enzyme Activation; Enzymes; Fatty liver; Female; Hepatitis C; Hepatitis C, Chronic - complications; Hepatitis C, Chronic - metabolism; Hepatitis C, Chronic - pathology; Hepatitis C, Chronic - virology; Homeostasis; Humans; Hypertension; Laboratories; Leukocyte Count; Lipid Metabolism; Lipids; Liver; Liver cirrhosis; Liver Cirrhosis - blood; Liver Cirrhosis - etiology; Liver Cirrhosis - metabolism; Liver diseases; lysosomal acid lipase; Male; Middle Aged; Neutrophils; non-alcoholic fatty liver disease; Non-alcoholic Fatty Liver Disease - blood; Non-alcoholic Fatty Liver Disease - etiology; Non-alcoholic Fatty Liver Disease - metabolism; Non-alcoholic Fatty Liver Disease - pathology; Pancytopenia; Patients; Peripheral blood; Platelet Count; Population; steatohepatitis; steatosis; Sterol Esterase - metabolism; Subgroups; Triglycerides; Wolman Disease; Wolman Disease - complications; Young Adult; steatohepatitis; lysosomal acid lipase; non-alcoholic fatty liver disease; steatosis; liver cirrhosis
• ISSN: 1422-0067; 1661-6596; 1422-0067
• DOI: 10.3390/ijms18061134

Lysosomal acid lipase (LAL) is a key enzyme in lipid metabolism. Initial reports have suggested a role for a relative acquired LAL deficiency in non-alcoholic fatty liver disease (NAFLD)-however, it is still unclear whether this mechanism is specific for NAFLD. We aimed to determine LAL activity in a cohort of NAFLD subjects and in a control group of hepatitis C virus (HCV)-infected patients, investigating the role of liver cirrhosis. A total of 81 patients with a diagnosis of NAFLD, and 78 matched controls with HCV-related liver disease were enrolled. For each patient, LAL activity was determined on peripheral dried blood spots (DBS) and correlated with clinical and laboratory data. A subgroup analysis among cirrhotic patients was also performed. LAL activity is significantly reduced in NAFLD, compared to that in HCV patients. This finding is particularly evident in the pre-cirrhotic stage of disease. LAL activity is also correlated with platelet and white blood cell count, suggesting an analytic interference of portal-hypertension-induced pancytopenia on DBS-determined LAL activity. NAFLD is characterized by a specific deficit in LAL activity, suggesting a pathogenetic role of LAL. We propose that future studies on this topic should rely on tissue specific analyses, as peripheral blood tests are also influenced by confounding factors.