Underediting of GluR2 mRNA, a neuronal death inducing molecular change in sporadic ALS, does not occur in motor neurons in ALS1 or SBMA.

Abstract

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.

• Authors: Yukio Kawahara, Hui Sun, Kyoko Ito, Takuto Hideyama, Masashi Aoki, Gen Sobue, Shoji Tsuji, Shin Kwak
• Journal: Neuroscience research (Neurosci Res) Vol. 54 Issue 1 Pg. 11-4 (Jan 2006) ISSN: 0168-0102 [Print] Ireland
• PMID: 16225946 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• RNA, Messenger
• Receptors, AMPA
• SOD1 protein, human
• Sod1 protein, mouse
• Sod1 protein, rat
• Superoxide Dismutase
• Superoxide Dismutase-1
• glutamate receptor ionotropic, AMPA 2

Topics

• Aged
• Amyotrophic Lateral Sclerosis (genetics, pathology)
• Animals
• Animals, Genetically Modified
• Cell Death (genetics)
• Humans
• Male
• Mice
• Motor Neurons (pathology, physiology)
• Muscular Atrophy, Spinal (genetics, pathology)
• Mutation
• RNA Editing
• RNA, Messenger
• Receptors, AMPA (genetics)
• Reverse Transcriptase Polymerase Chain Reaction
• Spinal Cord (pathology)
• Superoxide Dismutase (genetics)
• Superoxide Dismutase-1