Modeling spastic paraplegia 4 with corticospinal motor neuron-enriched cortical organoids reveals genotype-phenotype and HDAC6-targetable pathology
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- Title
- Modeling spastic paraplegia 4 with corticospinal motor neuron-enriched cortical organoids reveals genotype-phenotype and HDAC6-targetable pathology
- Creators
- Neha Mohan - Drexel UniversitySkandha Ramakrishnan - Drexel UniversityXiaohuan Sun - Drexel UniversityYing Sun - Nantong UniversityTheresa Connors - Drexel UniversityVictor Chai - Drexel UniversityEmanuela Piermarini - Drexel UniversityPeter W Baas - Drexel UniversityJames Cai - Texas A&M UniversityMei Liu - Nantong UniversityLiang Qiang (Corresponding Author) - Drexel University
- Publication Details
- Cell reports (Cambridge), v 45(3), 117036
- Publisher
- Elsevier
- Number of pages
- 23
- Grant note
- Blair Foundation
We thank Angela Altadonna for assisting in breeding mice for the experiments and Larisa Ibric and Luisa Abadia for assisting with behavioral studies. We would like to thank the staff of the Columbia Stem Cell Initiative Stem Cell Core facility, under the leadership of Dr. Barbara Corneo at Columbia University Irving Medical Center for assisting with CRISPR-Cas9 editing for the work, including Dr. Grazia Iannello, Dr. Dario Sirabella, Dr. Achchhe Patel, Dr. He-manta Sarmah, and Ms. Ekaterina Lebayle. We also want to thank Dr. Joanne Mathiasen for providing robust discussion and insights into dosing tubastatin A in mice, as well as Dr. Matthias Kneussel for providing the spastin knockout mice for breeding to generate the dHet mouse. All schematics were generated using BioRender. This research was supported by National Institutes of Health (R01NS115977) , the 2020 SPF Foundation Grant, and a research grant funded by als FINDa CURE and HOP on a CURE to L.Q.; by National Natural Science Foundation of China to M.L. (32070725) ; and by National Institutes of Health (R01NS118177) to P.W.B. Additional funding was provided by the CureSPG4 Foundation, the Lilly and Blair Foundation, SPG4 Cure for Jack Laidlaw, and the Maurya Koduri Foundation.
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Neurobiology and Anatomy; College of Medicine
- Web of Science ID
- WOS:001703638800001
- Other Identifier
- 991022162822204721