Nf1 Loss and Ras Hyperactivation in Oligodendrocytes Induce NOS-Driven Defects in Myelin and Vasculature

@inproceedings{Mayes2013Nf1LA,
  title={Nf1 Loss and Ras Hyperactivation in Oligodendrocytes Induce NOS-Driven Defects in Myelin and Vasculature},
  author={Debra A. Mayes and Tilat A. Rizvi and Haley E Titus-Mitchell and Rachel Oberst and Georgianne M. Ciraolo and Charles V Vorhees and Andrew P. Robinson and Stephen D Miller and Jose A. Cancelas and Anat Stemmer-Rachamimov and Nancy Ratner},
  booktitle={Cell reports},
  year={2013}
}
Patients with neurofibromatosis type 1 (NF1) and Costello syndrome Rasopathy have behavioral deficits. In NF1 patients, these may correlate with white matter enlargement and aberrant myelin. To model these features, we induced Nf1 loss or HRas hyperactivation in mouse oligodendrocytes. Enlarged brain white matter tracts correlated with myelin decompaction, downregulation of claudin-11, and mislocalization of connexin-32. Surprisingly, non-cell-autonomous defects in perivascular astrocytes and… CONTINUE READING
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