{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Bradic I"],"funding":["Austrian Science Fund FWF","NNF Center for Basic Metabolic Research"],"pagination":["711-723"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11401563"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["3(6)"],"pubmed_abstract":["<h4>Background and aims</h4>Recent studies showed that patients suffering from lysosomal acid lipase deficiency (LAL-D) benefit from enzyme replacement therapy; however, liver histopathology improved in some but not all patients. We hypothesized that the pan-peroxisome proliferator-activated receptor agonist lanifibranor may have beneficial effects on liver inflammation in LAL knockout (Lal-/-) mice based on its promising results in alleviating liver inflammation in patients with metabolic dysfunction-associated steatohepatitis.<h4>Methods</h4>Female Lal-/- mice were daily gavaged with lanifibranor or vehicle for 21 days. The effects of the treatment were assessed by measuring body and organ weights, plasma lipids and lipoproteins, as well as hematological parameters, followed by liver pro"],"journal":["Gastro hep advances"],"pubmed_title":["Lanifibranor Reduces Inflammation and Improves Dyslipidemia in Lysosomal Acid Lipase-Deficient Mice."],"pmcid":["PMC11401563"],"funding_grant_id":["CBMR - 007","P 32400"],"pubmed_authors":["Akhmetshina A","Kuentzel KB","Habisch H","Deshmukh AS","Pirchheim A","Kratky D","Vujic N","Bradic I","Madl T","Henderson JD"],"additional_accession":[]},"is_claimable":false,"name":"Lanifibranor Reduces Inflammation and Improves Dyslipidemia in Lysosomal Acid Lipase-Deficient Mice.","description":"<h4>Background and aims</h4>Recent studies showed that patients suffering from lysosomal acid lipase deficiency (LAL-D) benefit from enzyme replacement therapy; however, liver histopathology improved in some but not all patients. We hypothesized that the pan-peroxisome proliferator-activated receptor agonist lanifibranor may have beneficial effects on liver inflammation in LAL knockout (Lal-/-) mice based on its promising results in alleviating liver inflammation in patients with metabolic dysfunction-associated steatohepatitis.<h4>Methods</h4>Female Lal-/- mice were daily gavaged with lanifibranor or vehicle for 21 days. The effects of the treatment were assessed by measuring body and organ weights, plasma lipids and lipoproteins, as well as hematological parameters, followed by liver pro","dates":{"release":"2024-01-01T00:00:00Z","publication":"2024","modification":"2026-04-21T03:29:52.104Z","creation":"2025-04-04T01:17:23.452Z"},"accession":"S-EPMC11401563","cross_references":{"pubmed":["39280921"],"doi":["10.1016/j.gastha.2024.05.006"]}}