First Author | Polyzos A | Year | 2016 |
Journal | Hum Mol Genet | Volume | 25 |
Issue | 9 | Pages | 1792-802 |
PubMed ID | 26908614 | Mgi Jnum | J:233020 |
Mgi Id | MGI:5780618 | Doi | 10.1093/hmg/ddw051 |
Citation | Polyzos A, et al. (2016) Mitochondrial targeting of XJB-5-131 attenuates or improves pathophysiology in HdhQ150 animals with well-developed disease phenotypes. Hum Mol Genet 25(9):1792-802 |
abstractText | Oxidative damage to mitochondria (MT) is a major mechanism for aging and neurodegeneration. We have developed a novel synthetic antioxidant, XJB-5-131, which directly targets MT, the primary site and primary target of oxidative damage. XJB-5-131 prevents the onset of motor decline in an HdhQ(150/150) mouse model for Huntington's disease (HD) if treatment starts early. Here, we report that XJB-5-131 attenuates or reverses disease progression if treatment occurs after disease onset. In animals with well-developed pathology, XJB-5-131 promotes weight gain, prevents neuronal death, reduces oxidative damage in neurons, suppresses the decline of motor performance or improves it, and reduces a graying phenotype in treated HdhQ(150/150) animals relative to matched littermate controls. XJB-5-131 holds promise as a clinical candidate for the treatment of HD. |