title: Protective potential of resveratrol against oxidative stress and apoptosis in Batten disease lymphoblast cells.
abstract: Batten disease (BD) is the most common form of a group of disorders called neuronal ceroid lipofuscinosis, which are caused by a CLN3 gene mutation. A variety of pathogenic lysosomal storage disorder mechanisms have been suggested such as oxidative stress, endoplasmic reticulum (ER) stress, and altered protein trafficking. Resveratrol, a stilbenoid found in red grape skin, is a potent antioxidant chemical. Recent studies have suggested that resveratrol may have a curative effect in many neurodegenerative diseases. Therefore, we investigated the activities of resveratrol at the levels of oxidative and ER stress and apoptosis factors using normal and BD lymphoblast cells. We report that the BD lymphoblast cells contained low-levels of superoxide dismutase-1 (SOD-1) due to the long-term stress of reactive oxygen species. However, when we treated the cells with resveratrol, SOD-1 increased to levels observed in normal cells. Furthermore, we investigated the expression of glucose-regulated protein 78 as an ER stress marker. BD cells underwent ER stress, but resveratrol treatment resolved the ER stress in a dose-dependent manner. We further demonstrated that the levels of apoptosis markers such as apoptosis induce factor, cytochrome c, and cleavage of poly (ADP)-ribose polymerase decreased following resveratrol treatment. Thus, we propose that resveratrol may have beneficial effects in patients with BD.
authors.affiliation: Department of Biotechnology, Hoseo University, 165, Baebang, Asan, Chungnam 336-795, Republic of Korea.
authors.forename: Dong-Ho
authors.initials: DH
authors.lastname: Yoon
authors.forename: Oh-Youn
authors.initials: OY
authors.lastname: Kwon
authors.forename: Ji Young
authors.initials: JY
authors.lastname: Mang
authors.forename: Myung Jin
authors.initials: MJ
authors.lastname: Jung
authors.forename: Do Yeon
authors.initials: DY
authors.lastname: Kim
authors.forename: Yoon Kyung
authors.initials: YK
authors.lastname: Park
authors.forename: Tae-Hwe
authors.initials: TH
authors.lastname: Heo
authors.forename: Sung-Jo
authors.initials: SJ
authors.lastname: Kim
mesh_terms: D017209:Apoptosis / Q000187:drug effects*; D015415:Biomarkers / Q000378:metabolism; D002478:Cells, Cultured; D019610:Cytoprotection; D015536:Down-Regulation / Q000187:drug effects; D004721:Endoplasmic Reticulum / Q000378:metabolism; D000091342:Endoplasmic Reticulum Chaperone BiP; D006360:Heat-Shock Proteins / Q000037:antagonists & inhibitors / Q000378:metabolism; D006801:Humans; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism / Q000473:pathology*; D018696:Neuroprotective Agents / Q000494:pharmacology*; D018384:Oxidative Stress / Q000187:drug effects*; D000077185:Resveratrol; D013267:Stilbenes / Q000494:pharmacology*; D013482:Superoxide Dismutase / Q000096:biosynthesis; D000072105:Superoxide Dismutase-1
chemical_list: D015415:Biomarkers; D000091342:Endoplasmic Reticulum Chaperone BiP; D006360:Heat-Shock Proteins; D018696:Neuroprotective Agents; C000606290:SOD1 protein, human; D013267:Stilbenes; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1; D000077185:Resveratrol