A single-chain Fv intrabody provides functional protection against the effects of mutant protein in an organotypic slice culture model of Huntington's disease.

Abstract excerpt

Huntington's disease (HD) is a progressive, hereditary, neurodegenerative disorder caused by an expanded polyglutamine tract in huntingtin protein, leading to misfolding and abnormal protein-protein interactions. Reducing the initial misfolding should lead to decreased pathogenesis. We show that malonate stress increases the number of dead or dying cells when organotypic slice cultures are transduced to express...

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