Peroxisome deficiency-induced ER stress and SREBP-2 pathway activation in the liver of newborn PEX2 knock-out mice.

  title={Peroxisome deficiency-induced ER stress and SREBP-2 pathway activation in the liver of newborn PEX2 knock-out mice.},
  author={Werner J Kovacs and Khanichi N. Charles and Katharina Manuela Walter and Janis E. Shackelford and Thomas M Wikander and Michael S. Richards and Steven J. Fliesler and Skaidrite K. Krisans and Phyllis L. Faust},
  journal={Biochimica et biophysica acta},
  volume={1821 6},
Disruption of the Pex2 gene leads to peroxisome deficiency and widespread metabolic dysfunction. We previously demonstrated that peroxisomes are critical for maintaining cholesterol homeostasis, using peroxisome-deficient Pex2(-/-) mice on a hybrid Swiss Webster×129S6/SvEv (SW/129) genetic background. Peroxisome deficiency activates hepatic endoplasmic reticulum (ER) stress pathways, leading to dysregulation of the endogenous sterol response mechanism. Herein, we demonstrate a more profound… CONTINUE READING
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