Publications

Featured Publication

Nature

Neural and behavioural state switching during hippocampal dentate spikes

Jordan S Farrell, Ernie Hwaun, Barna Dudok, Ivan Soltesz

Nature. Published online March 13, 2024. doi:10.1038/s41586-024-07192-8

DOI: 10.1038/s41586-024-07192-8

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2024

American Journal of Medical Genetics

Deletions in the CDKL5 5 untranslated region lead to CDKL5 deficiency disorder

Isabel Haviland, Ralph D. Hector, Lindsay C. Swanson, Aubrie Soucy Verran, Emma Sherrill, Zoë Frazier, AnneMarie M. Denny, Jenna Lucash, Bo Zhang, Holly A. Dubbs, Eric D. Marsh, Judith L. Weisenberg, Helen Leonard, Milena Crippa, Francesca Cogliati, Silvia Russo, Bernhard Suter, Rajsekar Rajaraman, Alan K. Percy, John M. Schreiber, Scott Demarest, Timothy A. Benke, Maya Chopra, Timothy W. Yu, Heather E. Olson.

Am J Med Genet A. 2024 Aug 28. doi: 10.1002/ajmg.a.63843. Epub ahead of print. PMID:39205479.

https://doi.org/10.1002/ajmg.a.63843

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Journal of Inherited Metabolic Disease

Gene replacement therapies for inherited disorders of neurotransmission: Current progress in succinic semialdehyde dehydrogenase deficiency

Henry H C Lee, Itay Tokatly Latzer, Mariarita Bertoldi, Guangping Gao, Phillip L Pearl, Mustafa Sahin, Alexander Rotenberg

J Inherit Metab Dis. 2024 Apr 6. doi: 10.1002/jimd.12735. Epub ahead of print. PMID: 38581234.

https://doi.org/10.1002/jimd.12735

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Neurobiology of Disease

ALDH5A1-deficient iPSC-derived excitatory and inhibitory neurons display cell type specific alterations

Santana Almansa A, Gable DL, Frazier Z, Sveden A, Quinlan A, Chopra M, Lewis SA, Kruer M, Poduri A, Srivastava S.

Neurobiol Dis. 2024 Jan;190:106386. doi: 10.1016/j.nbd.2023.106386. Epub 2023 Dec 16. PMID: 38110041.
https://doi.org/10.1016/j.nbd.2023.106386

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2023

Genetics in Medicine

Clinical variants paired with phenotype: A rich resource for brain gene curation

Maya Chopra, Juliann M Savatt, Taylor I Bingaman, Molly E Good, Alexis Morgan, Caitlin Cooney, Allison M Rossel, Bryanna VanHoute, Ineke Cordova, Sonal Mahida, Virginia Lanzotti, Dustin Baldridge, Christina A Gurnett, Joseph Piven, Heather Hazlett, Scott L Pomeroy, Mustafa Sahin, Philip R O Payne, Erin Rooney Riggs, John N Constantino; Brain Gene Registry Consortium

Genet Med. 2023 Dec 4:101035. doi: 10.1016/j.gim.2023.101035. Epub ahead of print. PMID: 38059438.
DOI: 10.1016/j.gim.2023.101035

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JAMA Pediatrics

Rare De Novo and Inherited Genes in Familial and Nonfamilial Pediatric Attention-Deficit/Hyperactivity Disorder

Anne B Arnett, Elizabeth Harstad, Mia O’Connell, Katheryn Hayes, Stephanie Brewster, William Barbaresi, Ryan N Doan

JAMA Pediatr. 2023 Nov 20:e234952.
https://doi.org/10.1001/jamapediatrics.2023.4952

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Therapeutic Advances in Rare Disease

Persistence of Autism Spectrum Disorder From Early Childhood Through School Age

Elizabeth Harstad, Ellen Hanson, Stephanie J Brewster, Rafael DePillis, Anna L Milliken, Gabriella Aberbach, Georgios Sideridis, William J Barbaresi

JAMA Pediatr. Published online October 2, 2023. 
DOI: 10.1001/jamapediatrics.2023.4003

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Therapeutic Advances in Rare Disease

Toward Representative Genomic Research: the Children’s Rare Disease Cohorts Experience

Zoë J. Frazier, Eurnestine Brown, Shira Rockowitz, Ted Lee, Bo Zhang, Abigail Sveden, Nancy L. Chamberlin, Kira A. Dies, Annapurna Poduri, Piotr Sliz and Maya Chopra on behalf of the CRDC Consortium

Therapeutic Advances in Rare Disease 2023 10.1177/263300402311814062.
https://doi.org/10.1177/26330040231181406

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GeneReviews

ANKRD17-Related Neurodevelopmental Syndrome Synonym: Chopra-Amiel-Gordon Syndrome (CAGS)

, Christopher Gordon, , Maya Chopra

Sveden A, Gordon CT, Amiel J, et al. ANKRD17-Related Neurodevelopmental Syndrome. 2022 Dec 22. In: Adam MP, Everman DB, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2022.
https://www.ncbi.nlm.nih.gov/books/NBK1116/

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Current Protocols

A Robust Pipeline for the Multi-Stage Accelerated Differentiation of Functional 3D Cortical Organoids From Human Pluripotent Stem Cells

Dosh Whye, Delaney Wood, Wardiya Afshar Saber, Erika M. Norabuena, Nina R. Makhortova, Mustafa Sahin, Elizabeth D. Buttermore

Current Protocols3, e641 (2023).
https://doi.org/10.1002/cpz1.641

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2022

Academic Press

Phenotyping of Human iPSC-derived Neurons: Patient-Driven Research

Edited by Elizabeth D. Buttermore

Phenotyping of Human iPSC-derived Neurons, Academic Press, 2023, ISBN 9780128222775, https://doi.org/10.1016/B978-0-12-822277-5.20001-1

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Frontiers in Psychiatry

16p13.11 deletion variants associated with neuropsychiatric disorders cause morphological and synaptic changes in induced pluripotent stem cell-derived neurons

Elizabeth D. Buttermore, Nickesha Anderson, Pin-Fang Chen, Nina R. Makhortova, Kristina H. Kim, Syed M. A. Wafa, Sean Dwyer, John M. Micozzi, Kellen D. Winden, Bo Zhang, Min-Joon Han, Robin J. Kleiman, Catherine A. Brownstein, Mustafa Sahin, and Joseph Gonzalez-Heydrich

Front. Psychiatry, 03 November 2022, Sec. Molecular Psychiatry, https://doi.org/10.3389/fpsyt.2022.924956

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JAMA Neurology

Molecular Diagnostic Yield of Exome Sequencing and Chromosomal Microarray in Cerebral Palsy: A Systematic Review and Meta-analysis

Siddharth Srivastava, Sara Lewis, Julie Cohen, Bo Zhang, Bhooma Aravamuthan, Maya Chopra, Mustafa Sahin, Michael Kruer, Annapurna Poduri

JAMA Neurology, 2022; Volume 2, Issue 10 e568, https://doi.org/10.1001/jamaneurol.2022.3549

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Molecular Therapy Methods & Clinical Development

GENE TARGET: A framework for evaluating Mendelian neurodevelopmental disorders for gene therapy

Maya Chopra, Meera E. Modi, Kira A. Dies, Nancy L. Chamberlin, Elizabeth D. Buttermore, Stephanie Jo Brewster, Lisa Prock, Mustafa Sahin

Molecular Therapy Methods & Clinical Development. Volume 27, 8 December 2022, Pages 32-46.
https://doi.org/10.1016/j.omtm.2022.08.007

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Current Protocols

Dynamic 3D Combinatorial Generation of hPSC-Derived Neuromesodermal Organoids With Diverse Regional and Cellular Identities

Dosh Whye, Delaney Wood, Kristina H. Kim, Cidi Chen, Nina Makhortova, Mustafa Sahin, Elizabeth D. Buttermore

Current Protocols, 2022; Volume 2, Issue 10 e568, https://doi.org/10.1002/cpz1.568

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Human Molecular Genetics

A randomized controlled trial of everolimus for neurocognitive symptoms in PTEN hamartoma tumor syndrome

Siddharth Srivastava, Booil Jo, Bo Zhang, Thomas Frazier, Anne Snow Gallagher, Fleming Peck, April R Levin, Sangeeta Mondal, Zetan Li, Rajna Filip-Dhima, Gregory Geisel, Kira A Dies, Amelia Diplock, Charis Eng, Rabi Hanna, Mustafa Sahin, Antonio Hardan, the Developmental Synaptopathies Consortium

Human Molecular Genetics, 2022; ddac111, https://doi.org/10.1093/hmg/ddac111

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The American Journal of Human Genetics

Neurodevelopmental copy-number variants: A roadmap to improving outcomes by uniting patient advocates, researchers, and clinicians for collective impact

Elizabeth Buttermore, Stormy Chamberlain, Jannine Cody, Gregory Costain, Louis Dang, Andrew DeWoody, Yssa DeWoody, Kira Dies, Evan Eichler, Santhosh Girirajan, Marie Gramm, Alycia Halladay, Dennis Lal, Matthew Lalli, Tess Levy, Glennis Logsdon, Daniel Lowenstein, Heather Mefford, Jennifer Mulle, Alysson Muotri, Melissa Murphy, Eduardo Perez Palma, Stefan Pinter, Rebecca Pollak, Ryan Purcell, Rodney Samaco, Bina Shah, Karun Singh, Joyce So, Maria Sundberg, Surabi Veeraragavan, Vanessa Vogel-Farley, Anthony Wynshaw-Boris

The American Journal of Human Genetics. Volume 109, Issue 8, 2021, Pages 1353-1365.
https://doi.org/10.1016/j.ajhg.2022.07.003

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Journal of Neurodevelopmental Disorders

Validation of a computational phenotype for finding patients eligible for genetic testing for pathogenic PTEN variants across three centers

Cartik Kothari, Siddharth Srivastava, Youssef Kousa, Rima Izem, Marcin Gierdalski, Dongkyu Kim, Amy Good, Kira A. Dies, Gregory Geisel, Hiroki Morizono, Vittorio Gallo, Scott L. Pomeroy, Gwenn A. Garden, Lisa Guay-Woodford, Mustafa Sahin & Paul Avillach

Journal of Neurodevelopmental Disorders 14, 24 (2022).
https://doi.org/10.1186/s11689-022-09434-0

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Molecular Therapy

Gene-based therapeutics for rare genetic neurodevelopmental psychiatric disorders

Beverly L. Davidson, Guangping Gao, Elizabeth Berry-Kravis, Allison M. Bradbury, Carsten Bönnemann, Joseph D. Buxbaum, Gavin R. Corcoran, Steven J. Gray, Heather Gray-Edwards, Robin J. Kleiman, Adam J. Shaywitz, Dan Wang, Huda Y. Zoghbi, Terence R. Flotte, Sitra Tauscher-Wisniewski, Cynthia J. Tifft, Mustafa Sahin

Molecular Therapy , Volume 30, Issue 7, 2022, Pages 2416-2428, ISSN 1525-0016,
https://doi.org/10.1016/j.ymthe.2022.05.014.

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2021

The American Journal of Human Genetics

Heterozygous ANKRD17 loss-of-function variants cause a syndrome with intellectual disability, speech delay, and dysmorphism

Maya Chopra, Meriel McEntagart, Jill Clayton-Smith, Konrad Platzer, Anju Shukla, Katta M. Girisha, Anupriya Kaur, Parneet Kaur, Rolph Pfundt, Hermine Veenstra-Knol, Grazia M.S. Mancini, Gerarda Cappuccio, Nicola Brunetti-Pierri, Fanny Kortüm, Maja Hempel, Jonas Denecke, Anna Lehman, Tjitske Kleefstra, Kyra E. Stuurman, Martina Wilke, Michelle L. Thompson, E. Martina Bebin, Emilia K. Bijlsma, Mariette J.V. Hoffer, Cacha Peeters-Scholte, Anne Slavotinek, William A. Weiss, Tiffany Yip, Ugur Hodoglugil, Amy Whittle, Janette diMonda, Juanita Neira, Sandra Yang, Amelia Kirby, Hailey Pinz, Rosan Lechner, Frank Sleutels, Ingo Helbig, Sarah McKeown, Katherine Helbig, Rebecca Willaert, Jane Juusola, Jennifer Semotok, Medard Hadonou, John Short, Naomi Yachelevich, Sajel Lala, Alberto Fernández-Jaen, Janvier Porta Pelayo, Chiara Klöckner, Susanne B. Kamphausen, Rami Abou Jamra, Maria Arelin, A. Micheil Innes, Anni Niskakoski, Sam Amin, Maggie Williams, Julie Evans, Sarah Smithson, Damian Smedley, Anna de Burca, Usha Kini, Martin B. Delatycki, Lyndon Gallacher, Alison Yeung, Lynn Pais, Michael Field, Ellenore Martin, Perrine Charles, Thomas Courtin, Boris Keren, Maria Iascone, Anna Cereda, Gemma Poke, Véronique Abadie, Christel Chalouhi, Padmini Parthasarathy, Benjamin J. Halliday, Stephen P. Robertson, Stanislas Lyonnet, Jeanne Amiel, Christopher T. Gordon

The American Journal of Human Genetics, Volume 108, Issue 6, 2021, Pages 1138-1150,ISSN 0002-9297, https://doi.org/10.1016/j.ajhg.2021.04.007.

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