Neurodegeneration caused by polyglutamine expansion is regulated by P-glycoprotein in Drosophila melanogaster.

ABSTRACT: Trinucleotide CAG repeat disorders are caused by expansion of polyglutamine (polyQ) domains in certain proteins leading to fatal neurodegenerative disorders and are characterized by accumulation of inclusion bodies in the neurons. Clearance of these inclusion bodies holds the key to improve the disease phenotypes, which affects basic cellular processes such as transcription, protein degradation and cell signaling. In the present study, we show that P-glycoprotein (P-gp), originally identified as a causative agent of multidrug-resistant cancer cells, plays an important role in ameliorating the disease phenotype. Using a Drosophila transgenic strain that expresses a stretch of 127 glutamine repeats, we demonstrate that enhancing P-gp levels reduces eye degeneration caused by expression of po

SUBMITTER: Yadav S 

PROVIDER: S-EPMC3813869 | biostudies-literature | 2013 Nov

REPOSITORIES: biostudies-literature