Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA.
Deficient RNA editing of the AMPA receptor subunit GluR2 at the Q/R site is a primary cause of neuronal death and recently has been reported to be a tightly linked etiological cause of motor neuron death in sporadic amyotrophic lateral sclerosis (ALS). We quantified the RNA editing efficiency of the GluR2 Q/R site in single motor neurons of rats transgenic for mutant human Cu/Zn-superoxide dismutase (SOD1) as well as patients with spinal and bulbar muscular atrophy (SBMA), and found that GluR2 mRNA was completely edited in all the motor neurons examined. It seems likely that the death cascade is different among the dying motor neurons in sporadic ALS, familial ALS with mutant SOD1 and SBMA.
Kawahara
Yukio
Y
Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Sun
Hui
H
Ito
Kyoko
K
Hideyama
Takuto
T
Aoki
Masashi
M
Sobue
Gen
G
Tsuji
Shoji
S
Kwak
Shin
S
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000473:pathology; D000818:Animals; D030801:Animals, Genetically Modified; D016923:Cell Death / Q000235:genetics*; D006801:Humans; D008297:Male; D051379:Mice; D009046:Motor Neurons / Q000473:pathology / Q000502:physiology*; D009134:Muscular Atrophy, Spinal / Q000235:genetics* / Q000473:pathology; D009154:Mutation; D017393:RNA Editing; D012333:RNA, Messenger; D018091:Receptors, AMPA / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013116:Spinal Cord / Q000473:pathology; D013482:Superoxide Dismutase / Q000235:genetics; D000072105:Superoxide Dismutase-1
D012333:RNA, Messenger; D018091:Receptors, AMPA; C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; C000606292:Sod1 protein, rat; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1; C104722:glutamate receptor ionotropic, AMPA 2