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C9orf72 Poly(Pr) Mediated Neurodegeneration Is Associated With Nucleolar Stress, M. E. Cicardi, J. H. Hallgren, D. Mawrie, K. Krishnamurthy, S. S. Markandaiah, A. T. Nelson, V. Kankate, E. N. Anderson, P. Pasinelli, U. B. Pandey, C. M. Eischen, D. Trotti
C9orf72 Poly(Pr) Mediated Neurodegeneration Is Associated With Nucleolar Stress, M. E. Cicardi, J. H. Hallgren, D. Mawrie, K. Krishnamurthy, S. S. Markandaiah, A. T. Nelson, V. Kankate, E. N. Anderson, P. Pasinelli, U. B. Pandey, C. M. Eischen, D. Trotti
Farber Institute for Neuroscience Faculty Papers
The ALS/FTD-linked intronic hexanucleotide repeat expansion in the C9orf72 gene is aberrantly translated in the sense and antisense directions into dipeptide repeat proteins, among which poly proline-arginine (PR) displays the most aggressive neurotoxicity in-vitro and in-vivo. PR partitions to the nucleus when heterologously expressed in neurons and other cell types. We show that by lessening the nuclear accumulation of PR, we can drastically reduce its neurotoxicity. PR strongly accumulates in the nucleolus, a nuclear structure critical in regulating the cell stress response. We determined that, in neurons, PR caused nucleolar stress and increased levels of the transcription factor p53. …
Automated Subfield Volumetric Analysis Of Amygdala, Hippocampus, And Thalamic Nuclei In Mesial Temporal Lobe Epilepsy, Arichena Manmatharayan, Michael Kogan, Caio Matias, Mashaal Syed, India Shelley, Amar Chinni, Kichang Kang, Kiran Talekar, Scott H. Faro, Feroze B. Mohamed, Ashwini Sharan, Chengyuan Wu, Mahdi Alizadeh
Automated Subfield Volumetric Analysis Of Amygdala, Hippocampus, And Thalamic Nuclei In Mesial Temporal Lobe Epilepsy, Arichena Manmatharayan, Michael Kogan, Caio Matias, Mashaal Syed, India Shelley, Amar Chinni, Kichang Kang, Kiran Talekar, Scott H. Faro, Feroze B. Mohamed, Ashwini Sharan, Chengyuan Wu, Mahdi Alizadeh
Department of Neurosurgery Faculty Papers
Purpose: Identifying relationships between clinical features and quantitative characteristics of the amygdala-hippocampal and thalamic subregions in mesial temporal lobe epilepsy (mTLE) may offer insights into pathophysiology and the basis for imaging prognostic markers of treatment outcome. Our aim was to ascertain different patterns of atrophy or hypertrophy in mesial temporal sclerosis (MTS) patients and their associations with postsurgical seizure outcomes. To assess this aim, this study is designed in 2 folds: (1) hemispheric changes within MTS group and (2) association with postsurgical seizure outcomes.
Methods and materials: 27 mTLE subjects with mesial temporal sclerosis (MTS) were scanned for conventional 3D …