Amyotrophic lateral sclerosis (ALS) is neurodegenerative disorder resulting from selective loss of both upper and lower motor neurons leading to progressive muscle weakness and paralysis. In the present study, we have used 1H-[13C]-NMR spectroscopy in conjunction with infusion of [1,6-13C2]glucose to evaluate neurometabolic activity in SOD1G37R mouse model of ALS. Our finding suggest reduced levels of glutamate, NAA, NAAG, and increased concentration of myo-inositol in spinal cord. More interestingly, the metabolic activity of glutamatergic and GABAergic neurons is decreased in the spinal cord, while it was increased in the cerebral cortex indicating strikingly different pathology in spinal cord and brain.
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