Litcius/Paper detail

Absence of Sac2/INPP5F enhances the phenotype of a Parkinson’s disease mutation of synaptojanin 1

Mian Cao, Daehun Park, Yumei Wu, Pietro De Camilli

2020Proceedings of the National Academy of Sciences52 citationsDOIOpen Access PDF

Abstract

Significance Extensive genetic studies have identified numerous genes whose mutations results on Parkinson’s disease (PD), including synaptojanin 1 (SJ1/Park20), a nerve terminal enriched protein that includes an inositol 4-phosphatase domain (Sac domain). In addition, many PD candidate genes have been identified by genome-wide association studies, but for most of these genes, the link to PD remains hypothetical. One such gene is Sac2/INPP5F, which, interestingly, also includes an inositol 4-phosphatase domain. While Sac2KO mice do not show obvious defects, we show a striking synthetic effect in mice of the KO of Sac2 and the Sac domain mutation of SJ1 found in PD patients. These findings support a synergistic role of SJ1 and Sac2 on a PI4P pool whose dysfunction results in PD.

Topics & Concepts

PhenotypeMutationDiseaseNeuroscienceGeneticsBiologyMedicineGeneInternal medicineCellular transport and secretionParkinson's Disease Mechanisms and TreatmentsLysosomal Storage Disorders Research