NRF2 Activation Restores Disease Related Metabolic Deficiencies in Olfactory Neurosphere-Derived Cells from Patients with Sporadic Parkinson's Disease

• Published in: PloS one Vol. 6; no. 7; p. e21907
• Main Authors: Cook, Anthony L., Vitale, Alejandra M., Ravishankar, Sugandha, Matigian, Nicholas, Sutherland, Greg T., Shan, Jiangou, Sutharsan, Ratneswary, Perry, Chris, Silburn, Peter A., Mellick, George D., Whitelaw, Murray L., Wells, Christine A., Mackay-Sim, Alan, Wood, Stephen A.
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 01.07.2011; Public Library of Science (PLoS)
• Subjects: 1-Phosphatidylinositol 3-kinase; Ablation; AKT protein; Analysis; Antioxidants; Biology; Case-Control Studies; Cell Line; Cell lines; Defects; Dehydrogenases; Disease control; Drosophila; Enzymes; Etiology; Female; Gene expression; Gene Silencing; Genetic engineering; Glutathione; Glutathione - metabolism; Humans; Hydrogen peroxide; Insects; Isothiocyanates; Kinases; Male; Medical research; Medicine; Metabolic rate; Metabolism; Movement disorders; Mucosa; Neuroblastoma; Neurodegenerative diseases; NF-E2-Related Factor 2 - deficiency; NF-E2-Related Factor 2 - genetics; NF-E2-Related Factor 2 - metabolism; Olfactory epithelium; Olfactory Mucosa - drug effects; Olfactory Mucosa - metabolism; Olfactory Mucosa - pathology; Oxidative stress; Parkinson Disease - metabolism; Parkinson Disease - pathology; Parkinson's disease; Parkinsons disease; Pathology; Patients; Physiological aspects; Proteins; Rodents; Salts; Signal transduction; Signal Transduction - drug effects; Signaling; siRNA; Stem cells; Stress response; Tetrazolium Salts - metabolism; Thiazoles - metabolism; Thiocyanates - pharmacology; Transcription factors; Queensland Australia; Australia

Without appropriate cellular models the etiology of idiopathic Parkinson's disease remains unknown. We recently reported a novel patient-derived cellular model generated from biopsies of the olfactory mucosa (termed olfactory neurosphere-derived (hONS) cells) which express functional and geneti...