Identifier |
20130211_nanos_sciplatform1_06 |
Title |
Progranulin Deficiency Causes TDP43 Mislocalization and Neuron Death in the Retina |
Creator |
Ward, Michael E; Taubes, Alice; Miller, Bruce; Seely, Bill; Gelfland, Jeff; Herl, Lauren; Farese, Robert V; Gan, Li; Green, Ari |
Subject |
OCT; Retinal Ganglion Cells; Dementia; TDP-43; Progranulin |
Description |
Neurodegeneration and cytoplasmic mislocalization of TDP43 are pathologic hallmarks of sporadic frontotemporal lobar dementia TDP-43 subtype (FTLD-TDP) and familial FTLD caused by GRN mutations. However, the relationship between progranulin (PGRN)-deficiency, TDP43 mislocalization, and neuronal death remains unclear. Though brains from PGRN-deficient mice display some pathologic features of FTLD, neither a robust neurodegenerative phenotype nor TDP43 redistribution occurs. As such, fundamental aspects of FTLD pathogenesis have been difficult to model in these mice. Because retinal abnormalities are early feature of other neurodegenerative diseases, suggesting that retinal neurons are an especially vulnerable cell population in the CNS, we investigated whether retinal pathology occurred in a mouse model of GRN-deficient FTLD. |
Date |
2013-02-11 |
Language |
eng |
Format |
application/pdf |
Format Creation |
application/pdf |
Type |
Text |
Source |
2013 North American Neuro-Ophthalmology Society Annual Meeting |
Relation is Part of |
NANOS 2013: Platform Presentations (Session I) |
Collection |
Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu |
Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
Holding Institution |
North American Neuro-Ophthalmology Association. NANOS Executive Office 5841 Cedar Lake Road, Suite 204, Minneapolis, MN 55416 |
Rights Management |
Copyright 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
ARK |
ark:/87278/s68m0gs9 |
Context URL |
The NANOS Annual Meeting Neuro-Ophthalmology Collection: https://novel.utah.edu/collection/NAM/toc/ |
Contributor Primary |
FitzGibbon, Edmond |
Contributor Secondary |
Lam, Byron L |
Setname |
ehsl_novel_nam |
ID |
183417 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s68m0gs9 |