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ProteomicsPronounced metabolic alterations and susceptibility to inflammation, fibrosis, and cancer in livers from lysosomal acid lipase-deficient mice
Lysosomal acid lipase (LAL) is the sole lysosomal enzyme responsible for the degradation of cholesteryl esters and triacylglycerols at acidic pH. Impaired LAL activity leads to LAL deficiency (LAL-D), a severe and fatal disease characterized by ectopic lysosomal lipid accumulation. Reduced LAL activ...
ORGANISM(S): Mus musculus (Mouse) 
2023-10-24 | PXD042478 | Pride
Lysosomal acid lipase (lal) Lal deficiency Liver Mouse Non-alcoholic fatty liver disease (nafld) 
ProteomicsOff-target effects of the lysosomal acid lipase inhibitors Lalistat-1 and Lalistat-2 on neutral lipid hydrolases
Lysosomal acid lipase (LAL) is the key enzyme of lysosomal lipid hydrolysis, which degrades cholesteryl esters (CE), triacylglycerols (TG), diacylglycerols (DG), and retinyl esters. The role of LAL in various cellular processes has mostly been studied in LAL-deficient (Lal-/-) mice, which share phen...
ORGANISM(S): Mus musculus (Mouse) 
2022-05-13 | PXD030672 | Pride
Lal  lipolysis  lipid hydrolase activity  atgl  hsl  mgl 
ProteomicsRegional differences in the small intestinal proteome of control mice and of mice lacking lysosomal acid lipase
Background: Despite recent studies investigating the involvement of single cells in regional differences of the small intestine (SI), the metabolic contribution of the duodenum, jejunum, and ileum to the overall intestinal metabolism is still unclear. Basic procedures and main findings: Using an unt...
ORGANISM(S): Mus musculus (Mouse) 
2024-02-20 | PXD048378 | Pride
Metabolism Proteomics Lal Small intestine Macrophages 
ProteomicsLanifibranor reduces inflammation and improves dyslipidemia in lysosomal acid lipase-deficient mice
Background & Aims Recent studies showed that patients suffering from lysosomal acid lipase-deficiency (LAL-D) benefit tremendously from enzyme replacement therapy (ERT), however, the outcomes on liver histology in treated patients were inconsistent. The promising results of the pan-PPAR agonist lani...
ORGANISM(S): Mus musculus (Mouse) 
2024-06-12 | PXD047957 | Pride
Pan-ppar agonist Proteomics Lipid metabolism Lysosomal acid lipase deficiency Liver inflammation Plasma lipoproteins Lanifibranor 
ProteomicsLoss of lysosomal acid lipase results in mitochondrial dysfunction and fiber switch in skeletal muscles of mice
Objective: Lysosomal acid lipase (LAL) is the only enzyme known to hydrolyze cholesteryl esters (CE) and triacylglycerols in lysosomes at an acidic pH. Despite the importance of lysosomal hydrolysis in skeletal muscle (SM), research in this area is limited. We hypothesized that LAL may play an impor...
ORGANISM(S): Mus musculus (Mouse) 
2024-01-26 | PXD045665 | Pride
Muscle proteomics Lal Lal deficiency Energy metabolism Lal-deficient mouse 
GenomicsRescue of lysosomal acid lipase deficiency in mice by AAV liver gene transfer
Rescue of lysosomal acid lipase deficiency in mice by AAV liver gene transfer
PRJNA1062373 | ENA
UnknownLAL (Lysosomal Acid Lipase) Promotes Reverse Cholesterol Transport In Vitro and In Vivo.
Not available
S-EPMC5920716 | biostudies-literature
GenomicsHepatic Lysosomal Acid Lipase Overexpression Worsens Hepatic Inflammation in Mice Fed a Western Diet
Hepatic Lysosomal Acid Lipase Overexpression Worsens Hepatic Inflammation in Mice Fed a Western Diet
PRJNA748030 | ENA
GenomicsANGPTL4 suppress clear cell renal cell carcinoma via inhibition lysosomal acid lipase
ANGPTL4 suppress clear cell renal cell carcinoma via inhibition lysosomal acid lipase
PRJNA1140650 | ENA
TranscriptomicsHepatic Lysosomal Acid Lipase Overexpression Worsens Hepatic Inflammation in Mice Fed a Western Diet
Non-alcoholic fatty liver disease (NAFLD) is characterized by the accumulation of lipid droplets (LD) in hepatocytes. NAFLD development and progression is associated with an increase in hepatic cholesterol and decreased autophagy and lipophagy flux. Previous studies have shown that the expression of...
ORGANISM(S): Mus musculus 
2021-11-03 | GSE180377 | GEO
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