MEK Inhibitor Treatment Promotes Retinal Ganglion Cell Preservation without Preventing Retrobulbar Demyelination in Neurofibromatosis Mice

To better understand the disparity frequently seen between optic pathway glioma (OPG) characteristics and visual loss among young children with neurofibromatosis type 1 (NF1), we extend our previous findings of anatomic, physiologic, and behavioral abnormalities in an established animal model. We now report differential effects of treatment targeting the MAPK/ERK cellular pathway: 1) preserving retinal ganglion cell (RGC) number, and yet 2) failing to ameliorate demyelination of the retrobulbar optic nerve

MEK Inhibitor Treatment Promotes Retinal Ganglion Cell Preservation without Preventing Retrobulbar Demyelination in Neurofibromatosis Mice

To better understand the disparity frequently seen between optic pathway glioma (OPG) characteristics and visual loss among young children with neurofibromatosis type 1 (NF1), we extend our previous findings of anatomic, physiologic, and behavioral abnormalities in an established animal model. We now report differential effects of treatment targeting the MAPK/ERK cellular pathway: 1) preserving retinal ganglion cell (RGC) number, and yet 2) failing to ameliorate demyelination of the retrobulbar optic nerve

MEK Inhibitor Treatment Promotes Retinal Ganglion Cell Preservation without Preventing Retrobulbar Demyelination in Neurofibromatosis Mice

To better understand the disparity frequently seen between optic pathway glioma (OPG) characteristics and visual loss among young children with neurofibromatosis type 1 (NF1), we extend our previous findings of anatomic, physiologic, and behavioral abnormalities in an established animal model. We now report differential effects of treatment targeting the MAPK/ERK cellular pathway: 1) preserving retinal ganglion cell (RGC) number, and yet 2) failing to ameliorate demyelination of the retrobulbar optic nerve

Physiologic Dysfunction Precedes Retinal Ganglion Cell Loss in Mice with Neurofibromatosis and Optic Pathway Gliomas

We report previously unrecognized developmental abnormalities of retinal ganglion cell (RGC) anatomy and physiology that may help explain a striking disparity in young children with neurofibromatosis type 1 (NF1) who develop optic pathway gliomas (OPGs): the degree of visual loss is inadequately predicted by tumor characteristics or treatment response. In an animal model, we find RGC loss in discrete sectors months after tumor formation, but also greatly disrupted physiology among surviving retinal ganglion cells (RGCs)