N
Nicolas Arbez
Researcher at Johns Hopkins University School of Medicine
Publications - 38
Citations - 2625
Nicolas Arbez is an academic researcher from Johns Hopkins University School of Medicine. The author has contributed to research in topics: Huntingtin & Neurodegeneration. The author has an hindex of 23, co-authored 36 publications receiving 2214 citations. Previous affiliations of Nicolas Arbez include Johns Hopkins University & Centre national de la recherche scientifique.
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Journal ArticleDOI
Induced Pluripotent Stem Cells from Patients with Huntington’s Disease : Show CAG Repeat-Expansion-Associated Phenotypes
Virginia B. Mattis,Soshana P. Svendsen,Allison D. Ebert,Clive N. Svendsen,Alvin R. King,Malcolm Casale,Sara T. Winokur,Gayani Batugedara,Marquis P. Vawter,Peter J. Donovan,Leslie F. Lock,Leslie M. Thompson,Yu Zhu,Elisa Fossale,Ranjit Singh Atwal,Tammy Gillis,Jayalakshmi S. Mysore,Jian Hong Li,Ihn Sik Seong,Yiping Shen,Xiaoli Chen,Vanessa C. Wheeler,Marcy E. MacDonald,James F. Gusella,Sergey S Akimov,Nicolas Arbez,Tarja A. Juopperi,Tamara Ratovitski,Jason H. Chiang,Woon Roung Kim,Eka Chighladze,Erin Watkin,Chun Zhong,Georgia Makri,Robert N. Cole,Russell L. Margolis,Hongjun Song,Guo Li Ming,Christopher A. Ross,Julia A. Kaye,Julia A. Kaye,Aaron C. Daub,Aaron C. Daub,Punita Sharma,Punita Sharma,Amanda R. Mason,Amanda R. Mason,Steven Finkbeiner,Steven Finkbeiner,Junying Yu,James A. Thomson,David Rushton,Stephen P. Brazier,Alysia Battersby,Amanda Redfern,Hsui Er Tseng,Alexander William John Harrison,Paul J. Kemp,Nicholas D. Allen,Marco Onorati,Valentina Castiglioni,Elena Cattaneo,Jamshid Arjomand +62 more
TL;DR: The generation and characterization of 14 induced pluripotent stem cell (iPSC) lines from HD patients and controls reveal CAG-repeat-expansion-associated gene expression patterns that distinguish patient lines from controls, and early onset versus late onset HD.
Journal ArticleDOI
Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets
Mali Jiang,Jiawei Wang,Jiawei Wang,Jinrong Fu,Lin Du,Hyunkyung Jeong,Tim West,Lan Xiang,Qi Peng,Zhipeng Hou,Huan Cai,Tamara Seredenina,Nicolas Arbez,Shanshan Zhu,Katherine Sommers,Jennifer Qian,Jiangyang Zhang,Susumu Mori,X. William Yang,Kellie L.K. Tamashiro,Susan Aja,Timothy H. Moran,Ruth Luthi-Carter,Bronwen Martin,Stuart Maudsley,Mark P. Mattson,Robert H. Cichewicz,Christopher A. Ross,David M. Holtzman,Dimitri Krainc,Wenzhen Duan +30 more
TL;DR: Krainc et al. as mentioned in this paper showed that mutant HTT proteins in mice can bind and inactivate the deacetylase enzyme SIRT1 and that SIRT 1 overexpression is protective in Huntington's disease mouse models.
Journal ArticleDOI
Mutant Huntingtin Disrupts the Nuclear Pore Complex
Jonathan C. Grima,J. Gavin Daigle,Nicolas Arbez,Kathleen C. Cunningham,Ke Zhang,Joseph Ochaba,Charlene Geater,Eva L. Morozko,Jennifer Stocksdale,Jenna C. Glatzer,Jacqueline T. Pham,Ishrat Ahmed,Qi Peng,Harsh Wadhwa,Olga Pletnikova,Juan C. Troncoso,Wenzhen Duan,Solomon H. Snyder,Laura P.W. Ranum,Leslie M. Thompson,Thomas E. Lloyd,Christopher A. Ross,Jeffrey D. Rothstein +22 more
TL;DR: This work evaluated the NPC and nucleocytoplasmic transport in multiple models of HD, including mouse and fly models, neurons transfected with mHTT, HD iPSC-derived neurons, and human HD brain regions, and revealed severe mislocalization and aggregation of NUPs and defective nucleocy toplasmi transport.
Journal ArticleDOI
trans-(-)-ε-Viniferin increases mitochondrial sirtuin 3 (SIRT3), activates AMP-activated protein kinase (AMPK), and protects cells in models of Huntington Disease.
Jinrong Fu,Jing Jin,Robert H. Cichewicz,Serena A. Hageman,Trevor K. Ellis,Lan Xiang,Qi Peng,Mali Jiang,Nicolas Arbez,Katelyn Hotaling,Christopher A. Ross,Wenzhen Duan +11 more
TL;DR: A novel role for mitochondrial SIRT3 is established in HD pathogenesis and a natural product that has potent neuroprotection in HD models is discovered, suggesting that increasing mitochondrial Sirt3 might be considered as a new therapeutic approach to counteract HD.
Journal ArticleDOI
Developmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and mice
Ryan G. Lim,Lisa Salazar,D Wilton,Alvin R. King,Jennifer Stocksdale,Delaram Sharifabad,Alice L Lau,Beth Stevens,Jack C. Reidling,Sara T. Winokur,Malcolm Casale,Leslie M. Thompson,Mónica Pardo,A Gerardo García Díaz-Barriga,Marco Straccia,Phil Sanders,Jordi Alberch,Josep M. Canals,Julia A. Kaye,Mariah Dunlap,Lisa Jo,Hanna May,Elliot Mount,Cliff Anderson-Bergman,Kelly Haston,Steven Finkbeiner,Amanda J. Kedaigle,Theresa A. Gipson,Ferah Yildirim,Christopher W. Ng,Pamela Milani,David E. Housman,Ernest Fraenkel,Nicholas D. Allen,Paul J. Kemp,Ranjit Singh Atwal,Marta Biagioli,James F. Gusella,Marcy E. MacDonald,Sergey S Akimov,Nicolas Arbez,Jacqueline Stewart,Christopher A. Ross,Virginia B. Mattis,Colton M Tom,Loren Ornelas,Anais Sahabian,Lindsay Lenaeus,Berhan Mandefro,Dhruv Sareen,Clive N. Svendsen +50 more
TL;DR: The data suggest that mutant huntingtin impairs neurodevelopmental pathways that could disrupt synaptic homeostasis and increase vulnerability to the pathologic consequence of expanded polyglutamine repeat-associated phenotypes in neural cells and of cognitive impairment and synaptic pathology in HD model R6/2 mice.