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Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation.
Lim RG, Al-Dalahmah O, Wu J, Gold MP, Reidling JC, Tang G, Adam M, Dansu DK, Park HJ, Casaccia P, Miramontes R, Reyes-Ortiz AM, Lau A, Hickman RA, Khan F, Paryani F, Tang A, Ofori K, Miyoshi E, Michael N, McClure N, Flowers XE, Vonsattel JP, Davidson S, Menon V, Swarup V, Fraenkel E, Goldman JE, Thompson LM. Lim RG, et al. Among authors: miramontes r. Nat Commun. 2022 Dec 21;13(1):7791. doi: 10.1038/s41467-022-35388-x. Nat Commun. 2022. PMID: 36543778 Free PMC article.
Aberrant Development Corrected in Adult-Onset Huntington's Disease iPSC-Derived Neuronal Cultures via WNT Signaling Modulation.
Smith-Geater C, Hernandez SJ, Lim RG, Adam M, Wu J, Stocksdale JT, Wassie BT, Gold MP, Wang KQ, Miramontes R, Kopan L, Orellana I, Joy S, Kemp PJ, Allen ND, Fraenkel E, Thompson LM. Smith-Geater C, et al. Among authors: miramontes r. Stem Cell Reports. 2020 Mar 10;14(3):406-419. doi: 10.1016/j.stemcr.2020.01.015. Epub 2020 Feb 27. Stem Cell Reports. 2020. PMID: 32109367 Free PMC article.
PIAS1 modulates striatal transcription, DNA damage repair, and SUMOylation with relevance to Huntington's disease.
Morozko EL, Smith-Geater C, Monteys AM, Pradhan S, Lim RG, Langfelder P, Kachemov M, Kulkarni JA, Zaifman J, Hill A, Stocksdale JT, Cullis PR, Wu J, Ochaba J, Miramontes R, Chakraborty A, Hazra TK, Lau A, St-Cyr S, Orellana I, Kopan L, Wang KQ, Yeung S, Leavitt BR, Reidling JC, Yang XW, Steffan JS, Davidson BL, Sarkar PS, Thompson LM. Morozko EL, et al. Among authors: miramontes r. Proc Natl Acad Sci U S A. 2021 Jan 26;118(4):e2021836118. doi: 10.1073/pnas.2021836118. Proc Natl Acad Sci U S A. 2021. PMID: 33468657 Free PMC article.
Huntington's disease mice and human brain tissue exhibit increased G3BP1 granules and TDP43 mislocalization.
Sanchez II, Nguyen TB, England WE, Lim RG, Vu AQ, Miramontes R, Byrne LM, Markmiller S, Lau AL, Orellana I, Curtis MA, Faull RLM, Yeo GW, Fowler CD, Reidling JC, Wild EJ, Spitale RC, Thompson LM. Sanchez II, et al. Among authors: miramontes r. J Clin Invest. 2021 Jun 15;131(12):e140723. doi: 10.1172/JCI140723. J Clin Invest. 2021. PMID: 33945510 Free PMC article.
Microglia do not restrict SARS-CoV-2 replication following infection of the central nervous system of K18-hACE2 transgenic mice.
Olivarria GM, Cheng Y, Furman S, Pachow C, Hohsfield LA, Smith-Geater C, Miramontes R, Wu J, Burns MS, Tsourmas KI, Stocksdale J, Manlapaz C, Yong WH, Teijaro J, Edwards R, Green KN, Thompson LM, Lane TE. Olivarria GM, et al. Among authors: miramontes r. bioRxiv [Preprint]. 2021 Nov 17:2021.11.15.468761. doi: 10.1101/2021.11.15.468761. bioRxiv. 2021. PMID: 34816260 Free PMC article. Updated. Preprint.
Microglia Do Not Restrict SARS-CoV-2 Replication following Infection of the Central Nervous System of K18-Human ACE2 Transgenic Mice.
Olivarria GM, Cheng Y, Furman S, Pachow C, Hohsfield LA, Smith-Geater C, Miramontes R, Wu J, Burns MS, Tsourmas KI, Stocksdale J, Manlapaz C, Yong WH, Teijaro J, Edwards R, Green KN, Thompson LM, Lane TE. Olivarria GM, et al. Among authors: miramontes r. J Virol. 2022 Feb 23;96(4):e0196921. doi: 10.1128/jvi.01969-21. Epub 2021 Dec 22. J Virol. 2022. PMID: 34935438 Free PMC article.
Answer ALS, a large-scale resource for sporadic and familial ALS combining clinical and multi-omics data from induced pluripotent cell lines.
Baxi EG, Thompson T, Li J, Kaye JA, Lim RG, Wu J, Ramamoorthy D, Lima L, Vaibhav V, Matlock A, Frank A, Coyne AN, Landin B, Ornelas L, Mosmiller E, Thrower S, Farr SM, Panther L, Gomez E, Galvez E, Perez D, Meepe I, Lei S, Mandefro B, Trost H, Pinedo L, Banuelos MG, Liu C, Moran R, Garcia V, Workman M, Ho R, Wyman S, Roggenbuck J, Harms MB, Stocksdale J, Miramontes R, Wang K, Venkatraman V, Holewenski R, Sundararaman N, Pandey R, Manalo DM, Donde A, Huynh N, Adam M, Wassie BT, Vertudes E, Amirani N, Raja K, Thomas R, Hayes L, Lenail A, Cerezo A, Luppino S, Farrar A, Pothier L, Prina C, Morgan T, Jamil A, Heintzman S, Jockel-Balsarotti J, Karanja E, Markway J, McCallum M, Joslin B, Alibazoglu D, Kolb S, Ajroud-Driss S, Baloh R, Heitzman D, Miller T, Glass JD, Patel-Murray NL, Yu H, Sinani E, Vigneswaran P, Sherman AV, Ahmad O, Roy P, Beavers JC, Zeiler S, Krakauer JW, Agurto C, Cecchi G, Bellard M, Raghav Y, Sachs K, Ehrenberger T, Bruce E, Cudkowicz ME, Maragakis N, Norel R, Van Eyk JE, Finkbeiner S, Berry J, Sareen D, Thompson LM, Fraenkel E, Svendsen CN, Rothstein JD. Baxi EG, et al. Among authors: miramontes r. Nat Neurosci. 2022 Feb;25(2):226-237. doi: 10.1038/s41593-021-01006-0. Epub 2022 Feb 3. Nat Neurosci. 2022. PMID: 35115730 Free PMC article.
An altered extracellular matrix-integrin interface contributes to Huntington's disease-associated CNS dysfunction in glial and vascular cells.
Hernandez SJ, Lim RG, Onur T, Dane MA, Smith R, Wang K, Jean GE, Reyes-Ortiz A, Devlin K, Miramontes R, Wu J, Casale M, Kilburn D, Heiser LM, Korkola JE, Van Vactor D, Botas J, Thompson-Peer KL, Thompson LM. Hernandez SJ, et al. Among authors: miramontes r. Hum Mol Genet. 2023 Apr 20;32(9):1483-1496. doi: 10.1093/hmg/ddac303. Hum Mol Genet. 2023. PMID: 36547263 Free PMC article.
Single-nuclei transcriptome analysis of Huntington disease iPSC and mouse astrocytes implicates maturation and functional deficits.
Reyes-Ortiz AM, Abud EM, Burns MS, Wu J, Hernandez SJ, McClure N, Wang KQ, Schulz CJ, Miramontes R, Lau A, Michael N, Miyoshi E, Van Vactor D, Reidling JC, Blurton-Jones M, Swarup V, Poon WW, Lim RG, Thompson LM. Reyes-Ortiz AM, et al. Among authors: miramontes r. iScience. 2022 Dec 6;26(1):105732. doi: 10.1016/j.isci.2022.105732. eCollection 2023 Jan 20. iScience. 2022. PMID: 36590162 Free PMC article.
CryoET reveals organelle phenotypes in huntington disease patient iPSC-derived and mouse primary neurons.
Wu GH, Smith-Geater C, Galaz-Montoya JG, Gu Y, Gupte SR, Aviner R, Mitchell PG, Hsu J, Miramontes R, Wang KQ, Geller NR, Hou C, Danita C, Joubert LM, Schmid MF, Yeung S, Frydman J, Mobley W, Wu C, Thompson LM, Chiu W. Wu GH, et al. Among authors: miramontes r. Nat Commun. 2023 Feb 8;14(1):692. doi: 10.1038/s41467-023-36096-w. Nat Commun. 2023. PMID: 36754966 Free PMC article.
55 results