Article
Decreased ceramide underlies mitochondrial dysfunction in Charcot-Marie-Tooth 2F.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Mar 2018
Schwartz Nicholas U, Linzer Ryan W, Truman Jean-Philip, Gurevich Mikhail, Hannun Yusuf A, Senkal Can E, Obeid Lina M
Abstract excerpt
Charcot-Marie-Tooth (CMT) disease is the most commonly inherited neurologic disorder, but its molecular mechanisms remain unclear. One variant of CMT, 2F, is characterized by mutations in heat shock protein 27 (Hsp27). As bioactive sphingolipids have been implicated in neurodegenerative diseases, we sought to determine if their dysregulation is involved in CMT. Here, we show that Hsp27 knockout mice demonstrated...
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