Transcription profiling of mouse embryonic fibroblasts from HtrA2 knockoutafter rotenone treatment. HtrA2 knockout mice have a movement disorder which may be similar to Parkinsons disease

ABSTRACT: Cellular stress responses can be activated following functional defects in organelles such as mitochondria and the endoplasmic reticulum. Mitochondrial dysfunction caused by loss of the serine protease HtrA2 leads to a progressive movement disorder in mice and has been linked to parkinsonian neurodegeneration in humans. Here we demonstrate that loss of HtrA2 results in transcriptional up-regulation of nuclear genes characteristic of the integrated stress response, including the transcription factor CHOP, selectively in the brain. We also show that loss of HtrA2 results in the accumulation of unfolded proteins in the mitochondria, defective mitochondrial respiration and enhanced production of reactive oxygen species that contribute to the induction of CHOP expression and to neuronal cell de

ORGANISM(S): Mus musculus

SUBMITTER: Kristina Klupsch 

PROVIDER: E-GEOD-13034 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress