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HTT Evolution and Brain Development

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Programmed Cells from Basic Neuroscience to Therapy

Part of the book series: Research and Perspectives in Neurosciences ((NEUROSCIENCE,volume 20))

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Abstract

Huntingtin (htt) is the 800 million-year-old 3,144 amino acid protein product of the Huntington’s disease (HD) gene, which carries a tri-nucleotide CAG repeat then translated into polyglutamine (polyQ) at an evolutionarily conserved NH2-terminal position in exon 1. The CAG triplet is polymorphic in the normal population, ranging from 9 to 32 repetitions. In humans, an expansion of the repeats to more than 35 causes HD, a fatal, genetically dominant neurodegenerative disorder (MacDonald et al., Cell 72:971–983, 1993).

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Authors and Affiliations

  1. Centre for Stem Cell Research, Università degli Studi di Milano, Via Balzaretti 9, Milan, 20133, Italy

    Chiara Zuccato & Elena Cattaneo

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  1. Chiara Zuccato
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  2. Elena Cattaneo
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Correspondence to Elena Cattaneo .

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Editors and Affiliations

  1. , Laboratory of Genetics, The Salk Institute f. Biological Studies, North Torrey Pines Road 10010, La Jolla, 92037, California, USA

    Fred H. Gage

  2. Fondation Ipsen, Quai George Gorse 65, Boulogne Billancourt, 92650, France

    Yves Christen

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Zuccato, C., Cattaneo, E. (2013). HTT Evolution and Brain Development. In: Gage, F., Christen, Y. (eds) Programmed Cells from Basic Neuroscience to Therapy. Research and Perspectives in Neurosciences, vol 20. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-36648-2_5

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