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Glutamate receptors: RNA editing and death of motor neurons
A defect in the editing of the messenger RNA encoding the GluR2 subunit of glutamate AMPA receptors in the spinal motor neurons of individuals affected by ALS will interfere with the correct functioning of the glutamate receptors and may be a contributory cause of neuronal death in ALS patients.
Calcium-permeable AMPA channels in neurodegenerative disease and ischemia
Reduction of GluR2 RNA editing, a molecular change that increases calcium influx through AMPA receptors, selective in the spinal ventral gray of patients with amyotrophic lateral sclerosis
The decrement of GluR2 mRNA editing efficiency is unique to the ventral gray of ALS cases and may be closely linked to the etiology of ALS.
EAAT4, a glutamate transporter with properties of a chloride channel, is predominantly localized in Purkinje cell dendrites, and forms parasagittal compartments in rat cerebellum.
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2
The results suggest a molecular link between reduced ADAR2 activity and TDP-43 pathology, which is found to be found in spinal motor neurons of patients with sporadic ALS.
Induced Loss of ADAR2 Engenders Slow Death of Motor Neurons from Q/R Site-Unedited GluR2
GluR2 Q/R site editing causes AMPA receptor-mediated death of motor neurons in genetically modified mice and shows a decline in motor function commensurate with the slow death of ADAR2-deficient motor neurons.
AMPA receptor‐mediated neuronal death in sporadic ALS
- S. Kwak, T. Hideyama, T. Yamashita, H. Aizawa
- BiologyNeuropathology : official journal of the Japanese…
- 1 April 2010
It is likely that an increase of the proportion of Q/R site‐unedited GluR2‐containing Ca2+‐permeable AMPA receptors initiates the death of motor neurons in sporadic ALS.
Deficient RNA editing of GluR2 and neuronal death in amyotropic lateral sclerosis
GluR2 underediting occurs in a disease specific and region selective manner and is likely that the molecular mechanism underlying the deficiency in RNA editing is a reduction in the activity of ADAR2, a double- strand RNA specific deaminase.
Human spinal motoneurons express low relative abundance of GluR2 mRNA: an implication for excitotoxicity in ALS
The first quantitative measurements of the expression profile of AMPA receptor subunits mRNAs in human single neurons are provided by means of quantitative RT–PCR with a laser microdissector, showing that among the AMPA subunits, GluR2 shared the vast majority throughout the neuronal subsets and tissues examined.