Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies.

Huntington's disease is a dominant autosomal neurodegenerative disorder caused by an expansion of polyglutamines in the huntingtin (Htt) protein, whose cellular function remains controversial. To gain insight into Htt function, we purified epitope-tagged Htt and identified Argonaute as associated proteins. Colocalization studies demonstrated Htt and Ago2 to be present in ...
P bodies, and depletion of Htt showed compromised RNA-mediated gene silencing. Mouse striatal cells expressing mutant Htt showed fewer P bodies and reduced reporter gene silencing activity compared with wild-type counterparts. These data suggest that the previously reported transcriptional deregulation in HD may be attributed in part to mutant Htt's role in post-transcriptional processes.
Mesh Terms:
Cytoplasmic Structures, Eukaryotic Initiation Factor-2, Fluorescent Antibody Technique, Indirect, Humans, MicroRNAs, Microscopy, Confocal, Nerve Tissue Proteins, Nuclear Proteins, RNA Interference
Proc. Natl. Acad. Sci. U.S.A.
Date: Aug. 05, 2008
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