{"title":"CLN3 clinches lysosomes in clearance of glycerophospholipids","authors":"Guang Lu, Han-Ming Shen","doi":"10.1093/lifemeta/loac029","DOIUrl":null,"url":null,"abstract":"\n CLN3 is a lysosomal transmembrane protein and loss of CLN3 mutation is known to cause a juvenile lethal neurodegenerative lysosomal storage disorder (LSD), called Batten disease. In a recent study published in Nature, Laqtom et al. reported a novel function of CLN3 in the clearance of glycerophospholipid from lysosomes via lysosomal efflux of glycerophosphodiesters (GPDs), not only establishing a deeper mechanistic understanding of Batten disease, also suggesting both the diagnostic and therapeutic potential of CLN3-GPDs in this type of neurodegenerative LSD.","PeriodicalId":74074,"journal":{"name":"Life metabolism","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2022-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Life metabolism","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1093/lifemeta/loac029","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
CLN3 is a lysosomal transmembrane protein and loss of CLN3 mutation is known to cause a juvenile lethal neurodegenerative lysosomal storage disorder (LSD), called Batten disease. In a recent study published in Nature, Laqtom et al. reported a novel function of CLN3 in the clearance of glycerophospholipid from lysosomes via lysosomal efflux of glycerophosphodiesters (GPDs), not only establishing a deeper mechanistic understanding of Batten disease, also suggesting both the diagnostic and therapeutic potential of CLN3-GPDs in this type of neurodegenerative LSD.