Huntington’s disease and ubiquitin

Huntington’s disease or Huntington’s chorea is a terrible dominantly inherited neurodegenerative disease. The basis of this problem is an accumulation of fragments of polyglutamine expanded protein in affected neurons. There is also in Huntington’s disease and Alzheimers an abnormal enrichment of ubiquitin . . Ubiquitin metabolism may be in disarray. Ubiquitin is widely found in cells and nuclei and undergoes an ATP dependent reaction with proteins with condensation of its terminus with lysine amino groups. Cyclin degradation in the control of the cell cycle is triggered by ubiquitination .
Huntington’s disease is thought to be secondary to quite wide spread changes in ubiquitin metabolism.
Bennett et al 2007 Global changes to the ubiquitin system in Huntingdon’s disease.

Martin Eastwood
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