Mitochondrial dysfunction in ALS

Article ID	Journal	Published Year	Pages	File Type
6286589	Progress in Neurobiology	2012	13 Pages	PDF

Abstract

âº There is abundant evidence of mitochondrial damage in amyotrophic lateral sclerosis. âº Mitochondria have multiple functions that are essential for motor neuron survival. âº Energy production, calcium handling and mitochondrial dynamics are interconnected. âº The final outcome of mitochondrial damage may be cell death. âº Mitochondria-targeted therapies may be beneficial for ALS patients.

Keywords

DMPO Grx2 DLP1 tdp43 SALS Fis1 SOD2 VDAC Drp1 SOD1 EAAT GLT-1 MPTP AMPA CCS GSSG GSH Cu,Zn superoxide dismutase opa1 ROS optic atrophy 1 amyotrophic lateral sclerosis Sporadic amyotrophic lateral sclerosis familial amyotrophic lateral sclerosis mitochondria permeability transition pore ALS fALS excitatory amino acid transporter electron transport chain Copper chaperone for superoxide dismutase Mn-Superoxide dismutase Mitochondria Motor neuron ETc dynamin-like protein 1 dynamin-related protein 1 voltage-dependent anion channel reduced glutathione oxidized glutathione glutamate transporter 1 Reactive oxygen species