Article
α-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington's disease.
Human molecular genetics - 1 Feb 2012
Tomás-Zapico Cristina, Díez-Zaera María, Ferrer Isidre, Gómez-Ramos Pilar, Morán María A, Miras-Portugal M Teresa, Díaz-Hernández Miguel, Lucas José J
Abstract excerpt
Huntington's disease (HD) is the most common of nine inherited neurological disorders caused by expanded polyglutamine (polyQ) sequences which confer propensity to self-aggregate and toxicity to their corresponding mutant proteins. It has been postulated that polyQ expression compromises the folding capacity of the cell which might affect other misfolding-prone proteins. α-Synuclein (α-syn) is a small...
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