Nataliia N. Gotmanova, Anna V. Bacheva
Huntingtin,
the main factor in Huntington’s disease development. main functions and
intracellular proteolysis
Abstract
Abstract. This review is devoted to the consideration of pathological
intracellular mechanisms characteristic of Huntington’s disease and the central
role of huntingtin protein in these processes. The features of mutant
huntingtin aggregates utilization by the ubiquitin-proteasome system and
autophagy, as well as the possibilities of polyglutamine-containing substrates
hydrolysis by proteasome are discussed.
Key words: Huntington’s disease, huntingtin, polyglutamine tract, proteasome,
autophagy, aggregates
Copyright (C) Chemistry Dept., Moscow State University, 2002
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