Ontology highlight
ABSTRACT:
SUBMITTER: Jung J
PROVIDER: S-EPMC2778376 | biostudies-literature | 2009 Dec
REPOSITORIES: biostudies-literature
Jung Joonil J Xu Kexiang K Lessing Derek D Bonini Nancy M NM
Human molecular genetics 20090925 24
Protein cleavage is a common feature in human neurodegenerative disease. Ataxin-3 protein with an expanded polyglutamine (polyQ) repeat causes spinocerebellar ataxia type-3 (SCA3), also called Machado-Joseph disease, and is cleaved in mammalian cells, transgenic mice and SCA3 patient brain tissue. However, the pathological significance of Ataxin-3 cleavage has not been carefully examined. To gain insight into the significance of Ataxin-3 cleavage, we developed a Drosophila SL2 cell-based model a ...[more]