Search Results - "Rothstein, Jeffrey D."

Current hypotheses for the underlying biology of amyotrophic lateral sclerosis by Rothstein, Jeffrey D.

“…The mechanisms involved in selective motor neuron degeneration in amyotrophic lateral sclerosis remain unknown more than 135 years after the disease was first…”
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Astrocyte Diversity: Current Insights and Future Directions by Westergard, Thomas, Rothstein, Jeffrey D.

“…Astrocytes make up 20–40% of glial cells within the central nervous system (CNS) and provide several crucial functions, ranging from metabolic and structural…”
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The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease by Haeusler, Aaron R., Donnelly, Christopher J., Rothstein, Jeffrey D.

“…Key Points Since its discovery as the most common genetic abnormality in familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD),…”
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Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies by Vucic, Steve, Rothstein, Jeffrey D, Kiernan, Matthew C

“…Highlights • The pathophysiological mechanisms underlying amyotrophic lateral sclerosis (ALS) are multifactorial. • Genetic factors and dysfunction of vital…”
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NG2 + CNS Glial Progenitors Remain Committed to the Oligodendrocyte Lineage in Postnatal Life and following Neurodegeneration by Kang, Shin H., Fukaya, Masahiro, Yang, Jason K., Rothstein, Jeffrey D., Bergles, Dwight E.

“…The mammalian CNS contains a ubiquitous population of glial progenitors known as NG2 + cells that have the ability to develop into oligodendrocytes and undergo…”
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Rodent Models of Amyotrophic Lateral Sclerosis by Philips, Thomas, Rothstein, Jeffrey D

“…Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease affecting upper and lower motor neurons in the central nervous system. Patients with ALS develop…”
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Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis by Kang, Shin H, Li, Ying, Fukaya, Masahiro, Lorenzini, Ileana, Cleveland, Don W, Ostrow, Lyle W, Rothstein, Jeffrey D, Bergles, Dwight E

“…Oligodendrocytes form myelin sheaths and provide metabolic support to axons. Using in vivo genetic fate tracing in a mouse model of amyotrophic lateral…”
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The C9orf72 repeat expansion disrupts nucleocytoplasmic transport by Zhang, Ke, Donnelly, Christopher J., Haeusler, Aaron R., Grima, Jonathan C., Machamer, James B., Steinwald, Peter, Daley, Elizabeth L., Miller, Sean J., Cunningham, Kathleen M., Vidensky, Svetlana, Gupta, Saksham, Thomas, Michael A., Hong, Ingie, Chiu, Shu-Ling, Huganir, Richard L., Ostrow, Lyle W., Matunis, Michael J., Wang, Jiou, Sattler, Rita, Lloyd, Thomas E., Rothstein, Jeffrey D.

“…The hexanucleotide repeat expansion (HRE) GGGGCC (G 4 C 2 ) in C9orf72 is the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal…”
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C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import by Hayes, Lindsey R, Duan, Lauren, Bowen, Kelly, Kalab, Petr, Rothstein, Jeffrey D

“…Disruption of nucleocytoplasmic transport is increasingly implicated in the pathogenesis of neurodegenerative diseases, including ALS caused by a…”
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RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia by Zu, Tao, Liu, Yuanjing, Bañez-Coronel, Monica, Reid, Tammy, Pletnikova, Olga, Lewis, Jada, Miller, Timothy M, Harms, Matthew B, Falchook, Annet E, Subramony, S H, Ostrow, Lyle W, Rothstein, Jeffrey D, Troncoso, Juan C, Ranum, Laura P W

“…The finding that a GGGGCC (G ₄C ₂) hexanucleotide repeat expansion in the chromosome 9 ORF 72 (C9ORF72) gene is a common cause of amyotrophic lateral sclerosis…”
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Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS by Coyne, Alyssa N, Baskerville, Victoria, Zaepfel, Benjamin L, Dickson, Dennis W, Rigo, Frank, Bennett, Frank, Lusk, C Patrick, Rothstein, Jeffrey D

“…Alterations in the components [nucleoporins (Nups)] and function of the nuclear pore complex (NPC) have been implicated as contributors to the pathogenesis of…”
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Mutant Huntingtin Disrupts the Nuclear Pore Complex by Grima, Jonathan C., Daigle, J. Gavin, Arbez, Nicolas, Cunningham, Kathleen C., Zhang, Ke, Ochaba, Joseph, Geater, Charlene, Morozko, Eva, Stocksdale, Jennifer, Glatzer, Jenna C., Pham, Jacqueline T., Ahmed, Ishrat, Peng, Qi, Wadhwa, Harsh, Pletnikova, Olga, Troncoso, Juan C., Duan, Wenzhen, Snyder, Solomon H., Ranum, Laura P.W., Thompson, Leslie M., Lloyd, Thomas E., Ross, Christopher A., Rothstein, Jeffrey D.

“…Huntington’s disease (HD) is caused by an expanded CAG repeat in the Huntingtin (HTT) gene. The mechanism(s) by which mutant HTT (mHTT) causes disease is…”
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G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD by Coyne, Alyssa N., Zaepfel, Benjamin L., Hayes, Lindsey, Fitchman, Boris, Salzberg, Yuval, Luo, En-Ching, Bowen, Kelly, Trost, Hannah, Aigner, Stefan, Rigo, Frank, Yeo, Gene W., Harel, Amnon, Svendsen, Clive N., Sareen, Dhruv, Rothstein, Jeffrey D.

“…Through mechanisms that remain poorly defined, defects in nucleocytoplasmic transport and accumulations of specific nuclear-pore-complex-associated proteins…”
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CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation by Cheng, Weiwei, Wang, Shaopeng, Zhang, Zhe, Morgens, David W., Hayes, Lindsey R., Lee, Soojin, Portz, Bede, Xie, Yongzhi, Nguyen, Baotram V., Haney, Michael S., Yan, Shirui, Dong, Daoyuan, Coyne, Alyssa N., Yang, Junhua, Xian, Fengfan, Cleveland, Don W., Qiu, Zhaozhu, Rothstein, Jeffrey D., Shorter, James, Gao, Fen-Biao, Bassik, Michael C., Sun, Shuying

“…Hexanucleotide GGGGCC repeat expansion in C9ORF72 is the most prevalent genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)…”
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MCT1 Deletion in Oligodendrocyte Lineage Cells Causes Late-Onset Hypomyelination and Axonal Degeneration by Philips, Thomas, Mironova, Yevgeniya A., Jouroukhin, Yan, Chew, Jeannie, Vidensky, Svetlana, Farah, Mohamed H., Pletnikov, Mikhail V., Bergles, Dwight E., Morrison, Brett M., Rothstein, Jeffrey D.

“…Oligodendrocytes (OLs) are important for myelination and shuttling energy metabolites lactate and pyruvate toward axons through their expression of…”
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A Helicase Unwinds Hexanucleotide Repeat RNA G‑Quadruplexes and Facilitates Repeat-Associated Non-AUG Translation by Liu, Honghe, Lu, Yu-Ning, Paul, Tapas, Periz, Goran, Banco, Michael T, Ferré-D’Amaré, Adrian R, Rothstein, Jeffrey D, Hayes, Lindsey R, Myong, Sua, Wang, Jiou

“…The expansion of a hexanucleotide repeat GGGGCC (G4C2) in the C9orf72 gene is the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal…”
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Nuclear pore dysfunction and disease: a complex opportunity by Fare, Charlotte M, Rothstein, Jeffrey D

“…The separation of genetic material from bulk cytoplasm has enabled the evolution of increasingly complex organisms, allowing for the development of…”
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The blood-brain barrier: an engineering perspective by Wong, Andrew D, Ye, Mao, Levy, Amanda F, Rothstein, Jeffrey D, Bergles, Dwight E, Searson, Peter C

“…It has been more than 100 years since Paul Ehrlich reported that various water-soluble dyes injected into the circulation did not enter the brain. Since…”
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Molecular comparison of GLT1+ and ALDH1L1+ astrocytes in vivo in astroglial reporter mice by Yang, Yongjie, Vidensky, Svetlana, Jin, Lin, Jie, Chunfa, Lorenzini, Ileana, Frankl, Miriam, Rothstein, Jeffrey D.

“…Astrocyte heterogeneity remains largely unknown in the CNS due to lack of specific astroglial markers. In this study, molecular identity of in vivo astrocytes…”
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Absence of Survival and Motor Deficits in 500 Repeat C9ORF72 BAC Mice by Mordes, Daniel A., Morrison, Brett M., Ament, Xanthe H., Cantrell, Christopher, Mok, Joanie, Eggan, Pierce, Xue, Carolyn, Wang, Jin-Yuan, Eggan, Kevin, Rothstein, Jeffrey D.

“…A hexanucleotide repeat expansion at C9ORF72 is the most common genetic cause of amyotrophic lateral sclerosis (ALS)/frontotemporal dementia (FTD). Initial…”
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