摘要
Dear Editor,Glycosphingolipid(GSL)catabolism is strictly regulated in a sequential manner by lysosomal hydrolases with the help of lipid-binding proteins.Impairments of the catabolic pathway cause the onset of lysosomal storage diseases(LsDs),which are characterized by the aberrant accumulation of GSLs(Hannun and Obeid 2018;Breiden and Sandhoff 2019).The primary LSDs occur through the well-known mechanism of inherited defects in genes encoding the catabolic enzymes(or their related cofactors)which reside in the lumen of lysosomes(Hannun and Obeid 2018;Breiden and Sandhoff 2019).However,in some LSDs,the inherited mutations affect other proteins,such as those residing in the lysosomal membrane;these mutations are associated with defects in traffcking and fusion in the endocytic system(Platt et al.2012).However,the non-lumenal lysosomal membrane proteins involved in GSL metabolism remain to be identified.
基金
supported by grants from the Ministry of Science and Technology of the People's Republic of China(2021YFA0804800)
Tsinghua University Initiative Scientific Research Program(2021Z11JCQ016)
the National Natural Science Foundation of China(82088102,31625014).
作者简介
Yun Hong,contributed equally to this work;Zheng Tian,contributed equally to this work;Correspondence:Yiguo Wang,yiguo@mail.tsinghua.edu.cn。
