Expanded ATXN1 alters transcription and calcium signaling in SCA1 human motor neurons differentiated from induced pluripotent stem cells

Authors: Carrie Sheeler, Emmanuel Labrada, Lisa Duvick, Leslie M. Thompson, Ying Zhang, Harry T. Orr, Marija Cvetanovic

Source: Neurobiology of Disease, Vol 201, Iss , Pp 106673- (2024)

Subject Terms: SCA1, Motor neurons, iPSCs, Transcription, Calcium signaling, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S0969996124002730; https://doaj.org/toc/1095-953X

Access URL: https://doaj.org/article/340115b17b0e415f9876648f335e0074

Disease related changes in ATAC-seq of iPSC-derived motor neuron lines from ALS patients and controls

Authors: Stanislav Tsitkov, Kelsey Valentine, Velina Kozareva, Aneesh Donde, Aaron Frank, Susan Lei, the Answer ALS Consortium, Jennifer E. Van Eyk, Steve Finkbeiner, Jeffrey D. Rothstein, Leslie M. Thompson, Dhruv Sareen, Clive N. Svendsen, Ernest Fraenkel

Source: Nature Communications, Vol 15, Iss 1, Pp 1-15 (2024)

Subject Terms: Science

File Description: electronic resource

Relation: https://doaj.org/toc/2041-1723

Access URL: https://doaj.org/article/99c7b8055b0b402a8beedb66a340ca58

Identifying dysregulated regions in amyotrophic lateral sclerosis through chromatin accessibility outliers

Authors: Muhammed Hasan Çelik, Julien Gagneur, Ryan G. Lim, Jie Wu, Leslie M. Thompson, Xiaohui Xie

Source: HGG Advances, Vol 5, Iss 3, Pp 100318- (2024)

Subject Terms: Chromatin Accessibility, Outlier Detection, Aberrant Gene Expression, Rare Disease, Amyotrophic Lateral Sclerosis (ALS), Motor Neuron Disease, Genetics, QH426-470

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2666247724000575; https://doaj.org/toc/2666-2477

Access URL: https://doaj.org/article/3a8b144d826248da985431fd70085417

BDNF and TRiC-inspired reagent rescue cortical synaptic deficits in a mouse model of Huntington's disease

Authors: Yingli Gu, Alexander Pope, Charlene Smith, Christopher Carmona, Aaron Johnstone, Linda Shi, Xuqiao Chen, Sarai Santos, Claire Cecile Bacon-Brenes, Thomas Shoff, Korbin M. Kleczko, Judith Frydman, Leslie M. Thompson, William C. Mobley, Chengbiao Wu

Source: Neurobiology of Disease, Vol 195, Iss , Pp 106502- (2024)

Subject Terms: Huntington's disease, BACHD, Brain-derived neurotrophic factor, Synapse, Neuronal activity, Multi-array electrode, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S0969996124001013; https://doaj.org/toc/1095-953X

Access URL: https://doaj.org/article/9bef532de4e646788a3015cb4732bb13

CryoET reveals organelle phenotypes in huntington disease patient iPSC-derived and mouse primary neurons

Authors: Gong-Her Wu, Charlene Smith-Geater, Jesús G. Galaz-Montoya, Yingli Gu, Sanket R. Gupte, Ranen Aviner, Patrick G. Mitchell, Joy Hsu, Ricardo Miramontes, Keona Q. Wang, Nicolette R. Geller, Cathy Hou, Cristina Danita, Lydia-Marie Joubert, Michael F. Schmid, Serena Yeung, Judith Frydman, William Mobley, Chengbiao Wu, Leslie M. Thompson, Wah Chiu

Source: Nature Communications, Vol 14, Iss 1, Pp 1-20 (2023)

Subject Terms: Science

File Description: electronic resource

Relation: https://doaj.org/toc/2041-1723

Access URL: https://doaj.org/article/27dd4928dbf643889186e8bd55e8a97e

Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation

Authors: Ryan G. Lim, Osama Al-Dalahmah, Jie Wu, Maxwell P. Gold, Jack C. Reidling, Guomei Tang, Miriam Adam, David K. Dansu, Hye-Jin Park, Patrizia Casaccia, Ricardo Miramontes, Andrea M. Reyes-Ortiz, Alice Lau, Richard A. Hickman, Fatima Khan, Fahad Paryani, Alice Tang, Kenneth Ofori, Emily Miyoshi, Neethu Michael, Nicolette McClure, Xena E. Flowers, Jean Paul Vonsattel, Shawn Davidson, Vilas Menon, Vivek Swarup, Ernest Fraenkel, James E. Goldman, Leslie M. Thompson

Source: Nature Communications, Vol 13, Iss 1, Pp 1-23 (2022)

Subject Terms: Science

File Description: electronic resource

Relation: https://doaj.org/toc/2041-1723

Access URL: https://doaj.org/article/3ad164ee693049358f658929a36c0698

Single-nuclei transcriptome analysis of Huntington disease iPSC and mouse astrocytes implicates maturation and functional deficits

Authors: Andrea M. Reyes-Ortiz, Edsel M. Abud, Mara S. Burns, Jie Wu, Sarah J. Hernandez, Nicolette McClure, Keona Q. Wang, Corey J. Schulz, Ricardo Miramontes, Alice Lau, Neethu Michael, Emily Miyoshi, David Van Vactor, John C. Reidling, Mathew Blurton-Jones, Vivek Swarup, Wayne W. Poon, Ryan G. Lim, Leslie M. Thompson

Source: iScience, Vol 26, Iss 1, Pp 105732- (2023)

Subject Terms: Neurology, Omics, Science

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2589004222020053; https://doaj.org/toc/2589-0042

Access URL: https://doaj.org/article/3051cef5c5b24f3ebdab019b1d8be6cf

Gene expression and functional deficits underlie TREM2-knockout microglia responses in human models of Alzheimer’s disease

Authors: Amanda McQuade, You Jung Kang, Jonathan Hasselmann, Amit Jairaman, Alexandra Sotelo, Morgan Coburn, Sepideh Kiani Shabestari, Jean Paul Chadarevian, Gianna Fote, Christina H. Tu, Emma Danhash, Jorge Silva, Eric Martinez, Carl Cotman, G. Aleph Prieto, Leslie M. Thompson, Joan S. Steffan, Ian Smith, Hayk Davtyan, Michael Cahalan, Hansang Cho, Mathew Blurton-Jones

Source: Nature Communications, Vol 11, Iss 1, Pp 1-17 (2020)

Subject Terms: Science

File Description: electronic resource

Relation: https://doaj.org/toc/2041-1723

Access URL: https://doaj.org/article/2fc5155c4b834bdf97f0aa5db0218942

An integrated multi-omic analysis of iPSC-derived motor neurons from C9ORF72 ALS patients

Authors: Jonathan Li, Ryan G. Lim, Julia A. Kaye, Victoria Dardov, Alyssa N. Coyne, Jie Wu, Pamela Milani, Andrew Cheng, Terri G. Thompson, Loren Ornelas, Aaron Frank, Miriam Adam, Maria G. Banuelos, Malcolm Casale, Veerle Cox, Renan Escalante-Chong, J. Gavin Daigle, Emilda Gomez, Lindsey Hayes, Ronald Holewenski, Susan Lei, Alex Lenail, Leandro Lima, Berhan Mandefro, Andrea Matlock, Lindsay Panther, Natasha Leanna Patel-Murray, Jacqueline Pham, Divya Ramamoorthy, Karen Sachs, Brandon Shelley, Jennifer Stocksdale, Hannah Trost, Mark Wilhelm, Vidya Venkatraman, Brook T. Wassie, Stacia Wyman, Stephanie Yang, Jennifer E. Van Eyk, Thomas E. Lloyd, Steven Finkbeiner, Ernest Fraenkel, Jeffrey D. Rothstein, Dhruv Sareen, Clive N. Svendsen, Leslie M. Thompson, Hemali Phatnani, PhD, Justin Kwan, MD, Dhruv Sareen, PhD, James R. Broach, PhD, Zachary Simmons, MD, Ximena Arcila-Londono, MD, Edward B. Lee, MD, PhD, Vivianna M. Van Deerlin, MD, PhD, Neil A. Shneider, MD, PhD, Ernest Fraenkel, PhD, Lyle W. Ostrow, MD, PhD, Frank Baas, MD, PhD, Noah Zaitlen, PhD, James D. Berry, MD, MPH, Andrea Malaspina, MD, PhD, Pietro Fratta, MD, PhD, Gregory A. Cox, PhD, Leslie M. Thompson, PhD, Steve Finkbeiner, MD, PhD, Efthimios Dardiotis, MD, PhD, Timothy M. Miller, MD, PhD, Siddharthan Chandran, PhD, Suvankar Pal, MD, Eran Hornstein, MD, PhD, Daniel J. MacGowan, MD, Terry Heiman-Patterson, MD, Molly G. Hammell, PhD, Nikolaos.A. Patsopoulos, MD, PhD, Oleg Butovsky, PhD, Joshua Dubnau, PhD, Avindra Nath, MD, Robert Bowser, PhD, Matt Harms, MD, Mary Poss, DVM, PhD, Jennifer Phillips-Cremins, PhD, John Crary, MD, PhD, Nazem Atassi, MD, Dale J. Lange, MD, Darius J. Adams, MD, Leonidas Stefanis, MD, PhD, Marc Gotkine, MD, Robert H. Baloh, MD. PhD, Suma Babu, MBBS, MPH, Towfique Raj, PhD, Sabrina Paganoni, MD, PhD, Ophir Shalem, PhD, Colin Smith, MD, Bin Zhang, PhD, Brent Harris, MD, PhD, Iris Broce, PhD, Vivian Drory, MD, John Ravits, MD, Corey McMillan, PhD, Vilas Menon, PhD, Lani Wu, PhD, Steven Altschuler, PhD

Source: iScience, Vol 24, Iss 11, Pp 103221- (2021)

Subject Terms: Biological sciences, Neuroscience, Systems neuroscience, Systems biology, Omics, Science

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2589004221011895; https://doaj.org/toc/2589-0042

Access URL: https://doaj.org/article/b0aa201abf104ba08a6430dac01837c5

Author Correction: Gene expression and functional deficits underlie TREM2-knockout microglia responses in human models of Alzheimer’s disease

Authors: Amanda McQuade, You Jung Kang, Jonathan Hasselmann, Amit Jairaman, Alexandra Sotelo, Morgan Coburn, Sepideh Kiani Shabestari, Jean Paul Chadarevian, Gianna Fote, Christina H. Tu, Emma Danhash, Jorge Silva, Eric Martinez, Carl Cotman, G. Aleph Prieto, Leslie M. Thompson, Joan S. Steffan, Ian Smith, Hayk Davtyan, Michael Cahalan, Hansang Cho, Mathew Blurton-Jones

Source: Nature Communications, Vol 14, Iss 1, Pp 1-1 (2023)

Subject Terms: Science

File Description: electronic resource

Relation: https://doaj.org/toc/2041-1723

Access URL: https://doaj.org/article/d74b2e4cb1a84ae98fd95f8389c0a7c0

Cooperation of cell adhesion and autophagy in the brain: Functional roles in development and neurodegenerative disease

Authors: Sarah J. Hernandez, Gianna Fote, Andrea M. Reyes-Ortiz, Joan S. Steffan, Leslie M. Thompson

Source: Matrix Biology Plus, Vol 12, Iss , Pp 100089- (2021)

Subject Terms: Adhesion, Autophagy, Extracellular matrix, Huntington’s disease, Integrin, Neurodegenerative disease, Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2590028521000338; https://doaj.org/toc/2590-0285

Access URL: https://doaj.org/article/afd54e2517e9412daf298e30dcc82d7b

Aberrant Development Corrected in Adult-Onset Huntington's Disease iPSC-Derived Neuronal Cultures via WNT Signaling Modulation

Authors: Charlene Smith-Geater, Sarah J. Hernandez, Ryan G. Lim, Miriam Adam, Jie Wu, Jennifer T. Stocksdale, Brook T. Wassie, Maxwell Philip Gold, Keona Q. Wang, Ricardo Miramontes, Lexi Kopan, Iliana Orellana, Shona Joy, Paul J. Kemp, Nicholas D. Allen, Ernest Fraenkel, Leslie M. Thompson

Source: Stem Cell Reports, Vol 14, Iss 3, Pp 406-419 (2020)

Subject Terms: Medicine (General), R5-920, Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2213671120300369; https://doaj.org/toc/2213-6711

Access URL: https://doaj.org/article/ec7c074fe8934cefbe2687c75ff40f03

Calcium Dynamics in Astrocytes During Cell Injury

Authors: Nicole M. Wakida, Veronica Gomez-Godinez, Huayan Li, Jessica Nguyen, Edward K. Kim, Joseph L. Dynes, Shivashankar Othy, Alice L. Lau, Peng Ding, Linda Shi, Christopher Carmona, Leslie M. Thompson, Michael D. Cahalan, Michael W. Berns

Source: Frontiers in Bioengineering and Biotechnology, Vol 8 (2020)

Subject Terms: astrocyte, calcium, Salsa6f, phagocytosis, photolysis, laser nanosurgery, Biotechnology, TP248.13-248.65

File Description: electronic resource

Relation: https://www.frontiersin.org/article/10.3389/fbioe.2020.00912/full; https://doaj.org/toc/2296-4185

Access URL: https://doaj.org/article/ddebd47f3e8448bd8356530a827439ba

Human Neural Stem Cell Transplantation Rescues Functional Deficits in R6/2 and Q140 Huntington's Disease Mice

Authors: Jack C. Reidling, Aroa Relaño-Ginés, Sandra M. Holley, Joseph Ochaba, Cindy Moore, Brian Fury, Alice Lau, Andrew H. Tran, Sylvia Yeung, Delaram Salamati, Chunni Zhu, Asa Hatami, Carlos Cepeda, Joshua A. Barry, Talia Kamdjou, Alvin King, Dane Coleal-Bergum, Nicholas R. Franich, Frank M. LaFerla, Joan S. Steffan, Mathew Blurton-Jones, Charles K. Meshul, Gerhard Bauer, Michael S. Levine, Marie-Francoise Chesselet, Leslie M. Thompson

Source: Stem Cell Reports, Vol 10, Iss 1, Pp 58-72 (2018)

Subject Terms: Huntington's disease, neural stem cell, transplantation, R6/2 mice, Q140 mice, embryonic stem cells, Medicine (General), R5-920, Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2213671117304915; https://doaj.org/toc/2213-6711

Access URL: https://doaj.org/article/6a20e0b98a194e96bbd0862727f88f6d

The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin

Authors: Bo Wang, Li Zeng, Sean A. Merillat, Svetlana Fischer, Joseph Ochaba, Leslie M. Thompson, Sami J. Barmada, Kenneth M. Scaglione, Henry L. Paulson

Source: Neurobiology of Disease, Vol 109, Iss , Pp 127-136 (2018)

Subject Terms: Huntington's disease, Huntingtin, Ubiquitination, Ubiquitin-conjugating enzyme, Ube2W, Protein misfolding, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S096999611730222X; https://doaj.org/toc/1095-953X

Access URL: https://doaj.org/article/a0e2a6c0dc4745f68e4b209521fb211a

Huntington’s Disease iPSC-Derived Brain Microvascular Endothelial Cells Reveal WNT-Mediated Angiogenic and Blood-Brain Barrier Deficits

Authors: Ryan G. Lim, Chris Quan, Andrea M. Reyes-Ortiz, Sarah E. Lutz, Amanda J. Kedaigle, Theresa A. Gipson, Jie Wu, Gad D. Vatine, Jennifer Stocksdale, Malcolm S. Casale, Clive N. Svendsen, Ernest Fraenkel, David E. Housman, Dritan Agalliu, Leslie M. Thompson

Source: Cell Reports, Vol 19, Iss 7, Pp 1365-1377 (2017)

Subject Terms: Huntington’s disease, neurodegeneration, induced pluripotent stem cell, transcriptome, RNA sequencing, brain microvascular endothelial cell, BMEC, angiogenesis, blood-brain barrier, WNT signaling, epigenetics, Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2211124717304965; https://doaj.org/toc/2211-1247

Access URL: https://doaj.org/article/a4c8006ac0ea461986232304206450ef

Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia

Authors: Oliver H. Tam, Nikolay V. Rozhkov, Regina Shaw, Duyang Kim, Isabel Hubbard, Samantha Fennessey, Nadia Propp, Delphine Fagegaltier, Brent T. Harris, Lyle W. Ostrow, Hemali Phatnani, John Ravits, Josh Dubnau, Molly Gale Hammell, Justin Kwan, Dhruv Sareen, James R. Broach, Zachary Simmons, Ximena Arcila-Londono, Edward B. Lee, Vivianna M. Van Deerlin, Neil A. Shneider, Ernest Fraenkel, Frank Baas, Noah Zaitlen, James D. Berry, Andrea Malaspina, Pietro Fratta, Gregory A. Cox, Leslie M. Thompson, Steve Finkbeiner, Efthimios Dardiotis, Timothy M. Miller, Siddharthan Chandran, Suvankar Pal, Eran Hornstein, Daniel J. MacGowan, Terry Heiman-Patterson, Molly G. Hammell, Nikolaos.A. Patsopoulos, Oleg Butovsky, Joshua Dubnau, Avindra Nath, Robert Bowser, Matt Harms, Eleonora Aronica, Mary Poss, Jennifer Phillips-Cremins, John Crary, Nazem Atassi, Dale J. Lange, Darius J. Adams, Leonidas Stefanis, Marc Gotkine, Robert Baloh, Suma Babu, Towfique Raj, Sabrina Paganoni, Ophir Shalem, Colin Smith, Bin Zhang

Source: Cell Reports, Vol 29, Iss 5, Pp 1164-1177.e5 (2019)

Subject Terms: Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S221112471931263X; https://doaj.org/toc/2211-1247

Access URL: https://doaj.org/article/aae3b9d6ae684f00ab702dbeb5c75e19

SUMO-2 and PIAS1 Modulate Insoluble Mutant Huntingtin Protein Accumulation

Authors: Jacqueline Gire O’Rourke, Jaclyn R. Gareau, Joseph Ochaba, Wan Song, Tamás Raskó, David Reverter, John Lee, Alex Mas Monteys, Judit Pallos, Lisa Mee, Malini Vashishtha, Barbara L. Apostol, Thomas Peter Nicholson, Katalin Illes, Ya-Zhen Zhu, Mary Dasso, Gillian P. Bates, Marian Difiglia, Beverly Davidson, Erich E. Wanker, J. Lawrence Marsh, Christopher D. Lima, Joan S. Steffan, Leslie M. Thompson

Source: Cell Reports, Vol 4, Iss 2, Pp 362-375 (2013)

Subject Terms: Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2211124713003264; https://doaj.org/toc/2211-1247

Access URL: https://doaj.org/article/2cae4460f6614c9f87bd7c29ab921075

Histone deacetylase (HDAC) inhibitors targeting HDAC3 and HDAC1 ameliorate polyglutamine-elicited phenotypes in model systems of Huntington's disease

Authors: Haiqun Jia, Judit Pallos, Vincent Jacques, Alice Lau, Bin Tang, Andrew Cooper, Adeela Syed, Judith Purcell, Yi Chen, Shefali Sharma, Gavin R. Sangrey, Shayna B. Darnell, Heather Plasterer, Ghazaleh Sadri-Vakili, Joel M. Gottesfeld, Leslie M. Thompson, James R. Rusche, J. Lawrence Marsh, Elizabeth A. Thomas

Source: Neurobiology of Disease, Vol 46, Iss 2, Pp 351-361 (2012)

Subject Terms: Neurodegenerative, Striatum, Disease, Epigenetic, Therapeutic, Chromatin, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S096999611200040X; https://doaj.org/toc/1095-953X

Access URL: https://doaj.org/article/ef4bd67eeef24038b1106af8a3c56e6d

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