Article
Peroxisome deficiency causes a complex phenotype because of hepatic SREBP/Insig dysregulation associated with endoplasmic reticulum stress.
The Journal of biological chemistry - 13 Mar 2009
Kovacs Werner J, Tape Khanichi N, Shackelford Janis E, Wikander Thomas M, Richards Michael J, Fliesler Steven J, Krisans Skaidrite K, Faust Phyllis L
Abstract excerpt
Regulation of hepatic cholesterol biosynthesis, lipogenesis, and insulin signaling intersect at the transcriptional level by control of SREBP and Insig genes. We previously demonstrated that peroxisome-deficient PEX2-/- mice activate SREBP-2 pathways but are unable to maintain normal cholesterol homeostasis. In this study, we demonstrate that oral bile acid treatment normalized hepatic and plasma cholesterol...
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