Ontology highlight
ABSTRACT:
SUBMITTER: Sano R
PROVIDER: S-EPMC2782904 | biostudies-literature | 2009 Nov
REPOSITORIES: biostudies-literature
Sano Renata R Annunziata Ida I Patterson Annette A Moshiach Simon S Gomero Elida E Opferman Joseph J Forte Michael M d'Azzo Alessandra A
Molecular cell 20091101 3
Mitochondria-associated ER membranes, or MAMs, define the sites of endoplasmic reticulum/mitochondria juxtaposition that control Ca(2+) flux between these organelles. We found that in a mouse model of the human lysosomal storage disease GM1-gangliosidosis, GM1-ganglioside accumulates in the glycosphingolipid-enriched microdomain (GEM) fractions of MAMs, where it interacts with the phosphorylated form of IP3 receptor-1, influencing the activity of this channel. Ca(2+) depleted from the ER is then ...[more]