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Updated: May 11, 2026

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
07:08

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

NUB1 snubs huntingtin toxicity

Rebecca Aron1, Andrey Tsvetkov, Steven Finkbeiner

  • 1Gladstone Institutes and Taube-Koret Center for Neurodegenerative Disease, San Francisco, California, USA. sfinkbeiner@gladstone.ucsf.edu

Nature Neuroscience
|April 27, 2013
PubMed
Summary

No abstract available in PubMed .

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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...