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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) 
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
UnknownLysosomal Acid Lipase Deficiency: Therapeutic Options.
Not available
S-EPMC7023879 | biostudies-literature
UnknownRecent insights into lysosomal acid lipase deficiency.
Not available
S-EPMC7614602 | 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
UnknownLysosomal Acid Lipase Deficiency: Genetics, Screening, and Preclinical Study.
Not available
S-EPMC9779616 | biostudies-literature
UnknownLysosomal acid lipase deficiency in pediatric patients: a scoping review.
Not available
S-EPMC9432115 | biostudies-literature
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 
UnknownLysosomal Acid Lipase Deficiency: Report of Five Cases across the Age Spectrum.
Not available
S-EPMC5828415 | biostudies-literature
UnknownCholesterol Ester Storage Disease in Two Field Spaniels With Lysosomal Acid Lipase Deficiency.
Not available
S-EPMC12380722 | biostudies-literature
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