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Correlation between frataxin expression and contractility revealed by in vitro Friedreich's ataxia cardiac tissue models engineered from human pluripotent stem cells.
Wong AO, Wong G, Shen M, Chow MZ, Tse WW, Gurung B, Mak SY, Lieu DK, Costa KD, Chan CW, Martelli A, Nabhan JF, Li RA. Wong AO, et al. Among authors: martelli a. Stem Cell Res Ther. 2019 Jul 8;10(1):203. doi: 10.1186/s13287-019-1305-y. Stem Cell Res Ther. 2019. PMID: 31286988 Free PMC article.
In vivo overexpression of frataxin causes toxicity mediated by iron-sulfur cluster deficiency.
Huichalaf C, Perfitt TL, Kuperman A, Gooch R, Kovi RC, Brenneman KA, Chen X, Hirenallur-Shanthappa D, Ma T, Assaf BT, Pardo I, Franks T, Monarski L, Cheng TW, Le K, Su C, Somanathan S, Whiteley LO, Bulawa C, Pregel MJ, Martelli A. Huichalaf C, et al. Among authors: martelli a. Mol Ther Methods Clin Dev. 2022 Feb 7;24:367-378. doi: 10.1016/j.omtm.2022.02.002. eCollection 2022 Mar 10. Mol Ther Methods Clin Dev. 2022. PMID: 35252470 Free PMC article.
A modified mouse model of Friedreich's ataxia with conditional Fxn allele homozygosity delays onset of cardiomyopathy.
Perfitt TL, Huichalaf C, Gooch R, Kuperman A, Ahn Y, Chen X, Ullas S, Hirenallur-Shanthappa D, Zhan Y, Otis D, Whiteley LO, Bulawa C, Martelli A. Perfitt TL, et al. Among authors: martelli a. Am J Physiol Heart Circ Physiol. 2024 Feb 1;326(2):H357-H369. doi: 10.1152/ajpheart.00496.2023. Epub 2023 Dec 1. Am J Physiol Heart Circ Physiol. 2024. PMID: 38038720
1,410 results