Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
Article Abstract:
The mechanisms involved in neurogeneration induced by the huntingtin gene in Hungtington disease (HD) were investigated. A murine cellular model of HD was conceived through the incorporation of huntingtin into cultured striatal neurons by transfection. It was revealed that neurodegeneration was prompted by huntingtin through apoptosis since it was involved in the translocation of the nucleus that triggered the nuclear apoptosis. Also, the induced neurogeneration relied on the presence of the mutant polyglutamine tract within the huntingtin.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 1998
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The ataxia-ome: Connecting disease proteins of the cerebellum
Article Abstract:
A protein-protein interaction network is described for inherited human ataxias, a group of diseases characterized by degeneration of cerebellar Purkinje cells. This protein interactome shows that the cerebellar ataxias not only share clinical and pathological characteristics but also have proteins, processes and pathways in common.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2006
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Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
Article Abstract:
Huntingtin helps in enhancing the vesicular transport of brain-derived neurotrophic factor (BDNF) along the microtubules, thus controlling the neurotrophic support and survival of neurons. Huntingtin assists in promoting the BDNF transport and the loss of the function of huntingtin may result in pathogenesis.
Publication Name: Cell
Subject: Biological sciences
ISSN: 0092-8674
Year: 2004
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