Article
Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment.
Proceedings of the National Academy of Sciences of the United States of America - 18 Aug 2009
Maynard Christa J, Böttcher Claudia, Ortega Zaira, Smith Ruben, Florea Bogdan I, Díaz-Hernández Miguel, Brundin Patrik, Overkleeft Hermen S, Li Jia-Yi, Lucas Jose J, Dantuma Nico P
Abstract excerpt
Aggregation-prone proteins have been suggested to overwhelm and impair the ubiquitin/proteasome system (UPS) in polyglutamine (polyQ) disorders, such as Huntington's disease (HD). Overexpression of an N-terminal fragment of mutant huntingtin (N-mutHtt), an aggregation-prone polyQ protein responsible for HD, obstructs the UPS in cellular models. Furthermore, based on the accumulation of polyubiquitin conjugates in...
Topics
- Animals
- Brain
- Disease Models, Animal
- Green Fluorescent Proteins
- Humans
- Huntington Disease
- Mice
- Mice, Inbred C57BL
- Mice, Transgenic
- Mutation