Mitochondrial dysfunction during loss of prohibitin 1 triggers Paneth cell defects and ileitis

What is it about?

Unhealthy mitochondria are associated with many diseases, but the role of mitochondrial stress in the pathophysiology of inflammatory bowel diseases is not well understood. It is known that Crohn's disease patients have defects in secretory cells called Paneth cells. We show that mice with genetic deletion of a mitochondrial protein called Prohibitin 1 in Paneth cells develop mitochondrial stress that leads to intestinal inflammation in the ileum. Treatment of these mice with an antioxidant targeted to the mitochondria called Mito-Tempo prevented Paneth cell defects and inflammation. These are the first results that present a causative role of mitochondrial stress in ileal inflammation. Mitochondrial-targeted therapeutics may have utility in Crohn's disease patients with Paneth cell defects.

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