Rotenone susceptibility phenotype in olfactory derived patient cells as a model of idiopathic Parkinson’s disease

ABSTRACT: Physiologically relevant cellular models are critical for understanding complex pathological processes. Parkinson’s disease (PD) is the second most common neurodegenerative disorder characterised by motor and non-motor symptoms. Several cellular models for PD have been developed to reproduce the abberant biochemical pathways associated with Parkinson’s disease such as oxidative stress, apoptosis, improper clearance of misfolded proteins and mitochondrial impairment. However, there is still a need for models that effectively recapitulate pathological phenotypes of PD. We previously reported that cells derived from the olfactory epithelium of idiopathic PD patients have altered cellular functions compared with age and gender-matched healthy controls. We also identified an underlying dysregulation of molecular pathways including the Nrf2 pathway in PD hONS cells. Here, we tested the hypothesis that hONS cells derived from PD patients are more vulnerable to extrinsic stressors. We identified that PD-hONS cells are particularly sensitive to mitochondrial and oxidative stress. Notably, PD hONS cells exposed to Rotenone undergo significant apoptosis, show reduced mitochondrial complex I activity and reduced induction of Heat Shock Protein HSP27.

ORGANISM(S): Homo sapiens

SUBMITTER: Nicholas Matigian 

PROVIDER: E-MTAB-4164 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress