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GenomicsCustom-designed lipid-based nanoparticles efficiently deliver mRNA to reverse the pathogenesis of LAL deficiency in a preclinical model
Custom-designed lipid-based nanoparticles efficiently deliver mRNA to reverse the pathogenesis of LAL deficiency in a preclinical model
PRJNA1181265 | ENA
TranscriptomicsCustom-designed lipid-based nanoparticles efficiently deliver mRNA to reverse the pathogenesis of LAL deficiency in a preclinical model
Lysosomal acid lipase (LAL) is a crucial enzyme responsible for the hydrolysis of triglycerides (TGs) and cholesteryl esters (CEs) in the lysosomes. LAL deficiency results in the extensive storage of CEs and TGs in the liver and the spleen, inducing hepatosplenomegaly, and can be a life-threatening ...
ORGANISM(S): Mus musculus 
2024-12-06 | GSE280933 | GEO
ProteomicsFrom LAL-D to MASLD: Insights into the role of LAL and Kupffer cells in liver inflammation and lipid metabolism
Metabolic dysfunction-associated steatotic liver disease (MASLD) is a prevalent liver pathology worldwide, closely associated with obesity and metabolic disorders. Increasing evidence suggests that macrophages play a crucial role in the development of MASLD. Several human studies have shown an inver...
ORGANISM(S): Mus musculus (Mouse) 
2024-12-01 | PXD056343 | Pride
Mouse Timstof pro 2 
UnknownImpact of (intestinal) LAL deficiency on lipid metabolism and macrophage infiltration.
Not available
S-EPMC10209539 | biostudies-literature
UnknownLysosomal Acid Lipase Deficiency in 23 Spanish Patients: High Frequency of the Novel c.966+2T>G Mutation in Wolman Disease.
Not available
S-EPMC5740053 | biostudies-literature
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 
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) 
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 
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 
UnknownFrom LAL-D to MASLD: Insights into the role of LAL and Kupffer cells in liver inflammation and lipid metabolism.
Not available
S-EPMC7618626 | biostudies-literature
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