Alterations in the properties of the cell membrane due to glycosphingolipid accumulation in a model of Gaucher disease.

ABSTRACT: Gaucher disease is a lysosomal storage disease characterized by the malfunction of glucocerebrosidase resulting in the accumulation of glucosylceramide and other sphingolipids in certain cells. Although the disease symptoms are usually attributed to the storage of undigested substrate in lysosomes, here we show that glycosphingolipids accumulating in the plasma membrane cause profound changes in the properties of the membrane. The fluidity of the sphingolipid-enriched membrane decreased accompanied by the enlargement of raft-like ordered membrane domains. The mobility of non-raft proteins and lipids was severely restricted, while raft-resident components were only mildly affected. The rate of endocytosis of transferrin receptor, a non-raft protein, was significantly retarded in Gaucher cel

SUBMITTER: Batta G 

PROVIDER: S-EPMC5760709 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature