Article
Loss of prion protein in a transgenic model of amyotrophic lateral sclerosis.
Molecular and cellular neurosciences - 1 Feb 2002
Dupuis Luc, Mbebi Corinne, Gonzalez de Aguilar José-Luis, Rene Frédérique, Muller André, de Tapia Marc, Loeffler Jean-Philippe
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a motor neuron degenerative disorder caused in a proportion of cases by missense mutations in the gene encoding Cu/Zn superoxide dismutase (Cu/Zn-SOD) which result in unknown, lethal enzymatic activity. Based on a differential screening approach, we show here that the gene encoding the cellular prion protein (PrP(C)) was specifically repressed in a transgenic model of ALS...
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