Article
Oligodendrocyte Nf1 Controls Aberrant Notch Activation and Regulates Myelin Structure and Behavior.
Cell reports - 18 Apr 2017
López-Juárez Alejandro, Titus Haley E, Silbak Sadiq H, Pressler Joshua W, Rizvi Tilat A, Bogard Madeleine, Bennett Michael R, Ciraolo Georgianne, Williams Michael T, Vorhees Charles V, Ratner Nancy
Abstract excerpt
The RASopathy neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant genetic disorders. In NF1 patients, neurological issues may result from damaged myelin, and mice with a neurofibromin gene (Nf1) mutation show white matter (WM) defects including myelin decompaction. Using mouse genetics, we find that altered Nf1 gene-dose in mature oligodendrocytes results in progressive myelin defects and...
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