Induction of parkinsonism-related proteins in the spinal motor neurons of transgenic mouse carrying a mutant SOD1 gene

Journal of Neuroscience Research
Nobutoshi MorimotoKoji Abe

Abstract

Amyotrophic lateral sclerosis is a progressive and fatal disease caused by selective death of motor neurons, and a number of these patients carry mutations in the superoxide dismutase 1 (SOD1) gene involved in ameliorating oxidative stress. Recent studies indicate that oxidative stress and disruption of mitochondrial homeostasis is a common mechanism for motor neuron degeneration in amyotrophic lateral sclerosis and the loss of midbrain dopamine neurons in Parkinson's disease. Therefore, the present study investigated the presence and alterations of familial Parkinson's disease-related proteins, PINK1 and DJ-1, in spinal motor neurons of G93ASOD1 transgenic mouse model of amyotrophic lateral sclerosis. Following onset of disease, PINK1 and DJ-1 protein expression increased in the spinal motor neurons. The activated form of p53 also increased and translocated to the nuclei of spinal motor neurons, followed by increased expression of p53-activated gene 608 (PAG608). This is the first report demonstrating that increased expression of PAG608 correlates with activation of phosphorylated p53 in spinal motor neurons of an amyotrophic lateral sclerosis model. These results provide further evidence of the profound correlations between s...Continue Reading

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Citations

Mar 12, 2011·Cell Death and Differentiation·A VilborgK G Wiman
Sep 11, 2010·Human Molecular Genetics·Alberto FerriMaria Teresa Carrì
Aug 14, 2012·Free Radical Biology & Medicine·Mahesh RamamoorthyVilhelm A Bohr
Oct 17, 2013·Journal of Neuropathology and Experimental Neurology·Sarah KnippenbergSusanne Petri
Mar 12, 2011·Journal of Chemical Neuroanatomy·Giorgio VivacquaLoredana D'Este
Aug 11, 2011·Progress in Neurobiology·Mauro Cozzolino, Maria Teresa Carrì
Jun 3, 2011·Journal of the Neurological Sciences·Nobutoshi MorimotoKoji Abe

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