Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease.

Many forms of neurodegeneration are associated with oxidative stress and mitochondrial dysfunction. Mitochondria are prominent targets of oxidative damage, however, it is not clear whether mitochondrial DNA (mtDNA) damage and/or its lack of repair are primary events in the delayed onset observed in Huntington's disease (HD). We hypothesize that an age-dependent increase in mtDNA damage contributes to mitochondrial dysfunction in HD.

Identification and characterization of RAD9B, a paralog of the RAD9 checkpoint gene.

RAD9 is an integral element of the PCNA-like HUS1-RAD1-RAD9 (9-1-1) complex that participates in genotoxin-induced CHK1 activation. We have identified a novel RAD9 paralog, dubbed RAD9B, in humans and mice. RAD9 and RAD9B share extensive amino acid homology throughout their entire sequences (36% identity, 48% similarity).