Ku70 Alleviates Neurodegeneration in Drosophila Models of Huntington's Disease

Ku70 Alleviates Neurodegeneration in Drosophila Models of Huntington's Disease

DNA damage accumulates in genome DNA during the long life of neurons, thus DNA damage repair is indispensable to keep normal functions of neurons. We previously reported that Ku70, a critical molecule for DNA double strand break (DSB) repair, is involved in the pathology of Huntington's disease (HD). Mutant huntingtin (Htt) impaired Ku70 function via direct interaction, and Ku70 supplementation...

Applying Bioinfomatics Strategies in Huntingtons Disease Research

Proteasome inhibition alleviates SNARE-dependent neurodegeneration.

Activation of the proteasomal degradation of misfolded proteins has been proposed as a therapeutic strategy for treating neurodegenerative diseases, but it is unclear whether proteasome dysfunction contributes to neurodegeneration. We tested the role of proteasome activity in neurodegeneration developed by mice lacking cysteine string protein-α (CSPα). Unexpectedly, we found that proteasome inh...

Drosophila models reveal novel insights into mechanisms underlying neurodegeneration

The SAGA chromatin modifying complex functions as a transcriptional coactivator for a large number of genes, and SAGA dysfunction has been linked to carcinogenesis and neurodegenerative disease. The protein complex is comprised of approximately 20 subunits, arranged in a modular fashion, and includes 2 enzymatic subunits: the Gcn5 acetyltransferase and the Non-stop deubiquitinase. As we learn m...

MRI Tz Hypointensities in basal ganglia of premanifest Huntingtons disease