UBQLN2 and HSP70 participate in Parkin-mediated mitophagy by facilitating outer mitochondrial membrane rupture

ABSTRACT: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two aging- related neurodegenerative diseases that share common key features, including aggregation of pathogenic proteins, dysfunction of mitochondria and impairment of autophagy. Mutations in ubiquilin 2 (UBQLN2), a shuttle protein in the ubiquitin-proteasome system (UPS), can cause ALS/FTD, but the mechanism underlying UBQLN2-mediated pathogenesis is still uncertain. Recent studies indicate that mitophagy, a selective form of autophagy which is crucial for mitochondrial quality control, is tightly associated with neurodegenerative diseases including Alzheimer's disease, Parkinson's disease and ALS. In this study, we show that after Parkin- dependent ubiquitination of damaged mitochondria, UBQLN2 is recruited to po

SUBMITTER: Qilian Ma 

PROVIDER: S-SCDT-10_15252-EMBR_202255859 | biostudies-other |

REPOSITORIES: biostudies-other