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Reduced C9orf72 expression exacerbates polyGR toxicity in patient iPSC-derived motor neurons and a Type I protein arginine methyltransferase inhibitor reduces that toxicity.
Front Cell Neurosci. 2023 Apr 17;17:1134090. doi: 10.3389/fncel.2023.1134090. eCollection 2023.
Front Cell Neurosci. 2023.
PMID: 37138766
Free PMC article.
Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia.
Mou Y, Dong Y, Chen Z, Denton KR, Duff MO, Blackstone C, Zhang SC, Li XJ.
Mou Y, et al. Among authors: denton kr.
Acta Neuropathol Commun. 2020 Dec 7;8(1):214. doi: 10.1186/s40478-020-01088-0.
Acta Neuropathol Commun. 2020.
PMID: 33287888
Free PMC article.
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Modeling Axonal Defects in Hereditary Spastic Paraplegia with Human Pluripotent Stem Cells.
Denton KR, Xu C, Shah H, Li XJ.
Denton KR, et al.
Front Biol (Beijing). 2016 Oct;11(5):339-354. doi: 10.1007/s11515-016-1416-0. Epub 2016 Sep 28.
Front Biol (Beijing). 2016.
PMID: 27956894
Free PMC article.
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Abnormal mitochondrial transport and morphology as early pathological changes in human models of spinal muscular atrophy.
Xu CC, Denton KR, Wang ZB, Zhang X, Li XJ.
Xu CC, et al. Among authors: denton kr.
Dis Model Mech. 2016 Jan;9(1):39-49. doi: 10.1242/dmm.021766. Epub 2015 Nov 19.
Dis Model Mech. 2016.
PMID: 26586529
Free PMC article.
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Modeling Axonal Phenotypes with Human Pluripotent Stem Cells.
Denton KR, Xu CC, Li XJ.
Denton KR, et al.
Methods Mol Biol. 2016;1353:309-21. doi: 10.1007/7651_2014_167.
Methods Mol Biol. 2016.
PMID: 25520289
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Pharmacologic rescue of axon growth defects in a human iPSC model of hereditary spastic paraplegia SPG3A.
Zhu PP, Denton KR, Pierson TM, Li XJ, Blackstone C.
Zhu PP, et al. Among authors: denton kr.
Hum Mol Genet. 2014 Nov 1;23(21):5638-48. doi: 10.1093/hmg/ddu280. Epub 2014 Jun 6.
Hum Mol Genet. 2014.
PMID: 24908668
Free PMC article.
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Loss of spastin function results in disease-specific axonal defects in human pluripotent stem cell-based models of hereditary spastic paraplegia.
Denton KR, Lei L, Grenier J, Rodionov V, Blackstone C, Li XJ.
Denton KR, et al.
Stem Cells. 2014 Feb;32(2):414-23. doi: 10.1002/stem.1569.
Stem Cells. 2014.
PMID: 24123785
Free PMC article.
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