Article Figures & Data
Figures
- Figure 1.
Neurofibromin signaling pathway regulation. The NF1 gene product, neurofibromin, functions as both a negative regulator of RAS activity (RAS GAP) and as a positive regulator of AC activity. Impaired neurofibromin function leads to increased RAS activation, resulting in high levels of RAF/MEK and AKT/mTOR signaling. Similarly, reduced neurofibromin function is associated with decreased cAMP levels and reduced protein kinase A (PKA) activity. GRD, GAP-related domain.
Tables
GEM strain Description CNS abnormality References Nf1+/− mice Mice with reduced Nf1 gene expression throughout Impaired learning, memory, and attention; increased astrogliosis Jacks et al., 1994; Silva et al., 1997; Gutmann et al., 1999; Rizvi et al., 1999 Nf1+/−Syn mice Mice with reduced Nf1 gene expression in hippocampal neurons Impaired learning, memory, and attention Cui et al., 2008 Nf1+/−GFAPCKO mice Nf1+/− mice with complete Nf1 loss in neuroglial progenitors Impaired learning, memory, and attention; optic glioma formation Brown et al., 2010a; Bajenaru et al., 2003 Nf1+/−; KRasGFAP mice Nf1+/− mice with KRas activation in neuroglial progenitors Optic glioma formation Dasgupta et al., 2005a Nf1;p53GFAPCKO mice Mice with Nf1 and p53 inactivation in neuroglial progenitors High-grade glioma formation Zhu et al., 2005b Nf1;p53; PtenGFAPCKO mice Mice with Nf1,p53, and Pten inactivation in neuroglial progenitors GBM formation Kwon et al., 2008 Nf1;p53; PtennestinCKO mice Mice with Nf1,p53, and Pten inactivation in neural stem cells GBM formation Chen et al., 2012
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Neurofibromatosis Type 1: Modeling CNS Dysfunction
