Ontology highlight
ABSTRACT:
SUBMITTER: Falk MJ
PROVIDER: S-EPMC3394513 | biostudies-literature | 2011 Jul
REPOSITORIES: biostudies-literature
Falk Marni J MJ Polyak Erzsebet E Zhang Zhe Z Peng Min M King Rhonda R Maltzman Jonathan S JS Okwuego Ezinne E Horyn Oksana O Nakamaru-Ogiso Eiko E Ostrovsky Julian J Xie Letian X LX Chen Jia Yan JY Marbois Beth B Nissim Itzhak I Clarke Catherine F CF Gasser David L DL
EMBO molecular medicine 20110608 7
Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellular mechanisms that cause the widely variable clinical findings. Here, we show that focal segmental glomerulopathy-like kidney disease in Pdss2 mutant animals with primary coenzyme Q (CoQ) deficiency is significantly ameliorated by oral treatment with probucol (1% w/w). Preventative effects in missense mutant mice are similar whether fed probucol from weaning or for 3 weeks prior to typical nephri ...[more]