2006Chinese Journal of Clinical NeurosciencesRequires access

Huntingtin and Huntington’s Disease

Gu Zhen

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Abstract

Many of human diseases,including several neurodegenerative disorders are thought to be caused by misfolding and aggregation of a disease-causing protein. Huntington’s disease (HD) occurs due to an expansion of polyglutamine tract in the protein named huntingtin. Mutant huntingtin forms inclusions in the nucleus and aggregates in the cytoplasm in neurons. We reviewed the mechanisms of protein misfolding in Huntington’s disease and recent progress in developing therapies for HD aimed at preventing or correcting misfolding and aggregation of mutant huntingtin.

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What this paper is about

Many of human diseases,including several neurodegenerative disorders are thought to be caused by misfolding and aggregation of a disease-causing protein. Huntington’s disease (HD) occurs due to an expansion of polyglutamine tract in the protein named huntingtin. Mutant huntingtin forms inclusions in the nucleus and aggregates in the cytoplasm in neurons. We reviewed the mechanisms of protein misfolding in Huntington’s disease and recent progress in developing therapies for HD aimed at preventing or correcting misfolding and aggregation of mutant huntingtin.

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Available abstract

Many of human diseases,including several neurodegenerative disorders are thought to be caused by misfolding and aggregation of a disease-causing protein. Huntington’s disease (HD) occurs due to an expansion of polyglutamine tract in the protein named huntingtin. Mutant huntingtin forms inclusions in the nucleus and aggregates in the cytoplasm in neurons. We reviewed the mechanisms of protein misfolding in Huntington’s disease and recent progress in developing therapies for HD aimed at preventing or correcting misfolding and aggregation of mutant huntingtin.

Key concepts: Huntingtin, Polyglutamine tract, Huntington's disease, Huntingtin Protein, Disease, Protein aggregation, Mutant, Neurodegeneration

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