Ontology highlight
ABSTRACT:
ORGANISM(S): Mus musculus
SUBMITTER: Marni Falk
PROVIDER: E-GEOD-27954 | biostudies-arrayexpress |
REPOSITORIES: biostudies-arrayexpress
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]