| Primary Identifier | IPR026847 | Type | Family |
| Short Name | VPS13 |
| description | Vacuolar protein sorting-associated protein 13 (VPS13) is involved in the delivery of proteins to the vacuole in vegetatively growing yeast []and also regulates membrane morphogenesis during sporulation [, ]. It mediates the transfer of lipids between membranes at organelle contact sites []and it is involved in mitochondrial lipid homeostasis [, ]. In humans, the hereditary disorders chorea acanthocytosis and Cohen syndrome are caused by mutations in members ofthis family that are orthologues of yeast VPS13 (VPS13A and VPS13B respectively). Human VPS13A binds phospholipids and is required for the formation or stabilization of ER-mitochondria contact sites which enable transfer of lipids between the ER and mitochondria [, , ]. It is also required for efficient lysosomal protein degradation []. |
