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Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.
Sharkey LM, Safren N, Pithadia AS, Gerson JE, Dulchavsky M, Fischer S, Patel R, Lantis G, Ashraf N, Kim JH, Meliki A, Minakawa EN, Barmada SJ, Ivanova MI, Paulson HL. Sharkey LM, et al. Proc Natl Acad Sci U S A. 2018 Oct 30;115(44):E10495-E10504. doi: 10.1073/pnas.1810522115. Epub 2018 Oct 17. Proc Natl Acad Sci U S A. 2018. PMID: 30333186 Free PMC article.
Modeling UBQLN2-mediated neurodegenerative disease in mice: Shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity.
Sharkey LM, Sandoval-Pistorius SS, Moore SJ, Gerson JE, Komlo R, Fischer S, Negron-Rios KY, Crowley EV, Padron F, Patel R, Murphy GG, Paulson HL. Sharkey LM, et al. Neurobiol Dis. 2020 Sep;143:105016. doi: 10.1016/j.nbd.2020.105016. Epub 2020 Jul 10. Neurobiol Dis. 2020. PMID: 32653673 Free PMC article.
Ubiquilin-2 differentially regulates polyglutamine disease proteins.
Gerson JE, Safren N, Fischer S, Patel R, Crowley EV, Welday JP, Windle AK, Barmada S, Paulson HL, Sharkey LM. Gerson JE, et al. Among authors: sharkey lm. Hum Mol Genet. 2020 Aug 29;29(15):2596-2610. doi: 10.1093/hmg/ddaa152. Hum Mol Genet. 2020. PMID: 32681165 Free PMC article.
Neuronal Puncta/Aggregate Formation by WT and Mutant UBQLN2.
Safren N, Sharkey LM, Barmada SJ. Safren N, et al. Among authors: sharkey lm. Methods Mol Biol. 2023;2551:561-573. doi: 10.1007/978-1-0716-2597-2_34. Methods Mol Biol. 2023. PMID: 36310225 Free PMC article.
Ubiquilin-2 regulates pathological alpha-synuclein.
Sandoval-Pistorius SS, Gerson JE, Liggans N, Ryou JH, Oak K, Li X, Negron-Rios KY, Fischer S, Barsh H, Crowley EV, Skinner ME, Sharkey LM, Barmada SJ, Paulson HL. Sandoval-Pistorius SS, et al. Among authors: sharkey lm. Sci Rep. 2023 Jan 6;13(1):293. doi: 10.1038/s41598-022-26899-0. Sci Rep. 2023. PMID: 36609661 Free PMC article.
43 results