Life Without Huntingtin. Normal Differentiation into FunctionalNeurons
نویسندگان
چکیده
منابع مشابه
Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice.
The N-terminus of Huntingtin, the protein encoded by the Huntington's disease gene, contains a stretch of polyglutamine residues that is expanded in Huntington's disease. The polyglutamine stretch is flanked by two conserved protein domains in vertebrates: an N1-17 domain, and a proline-rich region (PRR). The PRR can modulate the structure of the adjacent polyglutamine stretch, and is a binding...
متن کاملDisassociation of histone deacetylase-3 from normal huntingtin underlies mutant huntingtin neurotoxicity.
Huntington's disease (HD) is caused by a polyglutamine expansion within the huntingtin (Htt) protein. Both loss of function of normal Htt and gain of a toxic function by the polyglutamine-expanded mutant Htt protein have been proposed to be responsible for HD, although the molecular mechanisms involved are unclear. We show that Htt is a neuroprotective protein in both HD-related and unrelated m...
متن کاملHuntingtin Regulates Mammary Stem Cell Division and Differentiation
Little is known about the mechanisms of mitotic spindle orientation during mammary gland morphogenesis. Here, we report the presence of huntingtin, the protein mutated in Huntington's disease, in mouse mammary basal and luminal cells throughout mammogenesis. Keratin 5-driven depletion of huntingtin results in a decreased pool and specification of basal and luminal progenitors, and altered mamma...
متن کاملNormal Aging Modulates the Neurotoxicity of Mutant Huntingtin
Aging likely plays a role in neurodegenerative disorders. In Huntington's disease (HD), a disorder caused by an abnormal expansion of a polyglutamine tract in the protein huntingtin (Htt), the role of aging is unclear. For a given tract length, the probability of disease onset increases with age. There are mainly two hypotheses that could explain adult onset in HD: Either mutant Htt progressive...
متن کاملExpression of normal and mutant huntingtin in the developing brain.
Huntington's disease (HD) is caused by a genetic mutation that results in a polyglutamine expansion in huntingtin. The time course of neuronal loss in the HD striatum and other affected brain regions before the onset of symptoms is unknown. To determine the potential influence of huntingtin on brain development, we examined its expression in the developing mouse and in human control and HD brai...
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ژورنال
عنوان ژورنال: Journal of Neurochemistry
سال: 1999
ISSN: 0022-3042,1471-4159
DOI: 10.1046/j.1471-4159.1999.0721009.x