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A modified mouse model of Friedreich's ataxia with conditional Fxn allele homozygosity delays onset of cardiomyopathy.
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
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: gooch r.
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.
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4'-Phosphopantetheine and long acyl chain-dependent interactions are integral to human mitochondrial acyl carrier protein function.
Majmudar JD, Feng X, Fox NG, Nabhan JF, Towle T, Ma T, Gooch R, Bulawa C, Yue WW, Martelli A.
Majmudar JD, et al. Among authors: gooch r.
Medchemcomm. 2019 Jan 8;10(2):209-220. doi: 10.1039/c8md00489g. eCollection 2019 Feb 1.
Medchemcomm. 2019.
PMID: 30881609
Free PMC article.
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Mitigation of peroxynitrite-mediated nitric oxide (NO) toxicity as a mechanism of induced adaptive NO resistance in the CNS.
Bishop A, Gooch R, Eguchi A, Jeffrey S, Smallwood L, Anderson J, Estevez AG.
Bishop A, et al. Among authors: gooch r.
J Neurochem. 2009 Apr;109(1):74-84. doi: 10.1111/j.1471-4159.2009.05884.x. Epub 2009 Jan 13.
J Neurochem. 2009.
PMID: 19183270
Free PMC article.
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Perturbation of cellular proteostasis networks identifies pathways that modulate precursor and intermediate but not mature levels of frataxin.
Nabhan JF, Gooch RL, Piatnitski Chekler EL, Pierce B, Bulawa CE.
Nabhan JF, et al. Among authors: gooch rl.
Sci Rep. 2015 Dec 16;5:18251. doi: 10.1038/srep18251.
Sci Rep. 2015.
PMID: 26671574
Free PMC article.
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Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia.
Nabhan JF, Wood KM, Rao VP, Morin J, Bhamidipaty S, LaBranche TP, Gooch RL, Bozal F, Bulawa CE, Guild BC.
Nabhan JF, et al. Among authors: gooch rl.
Sci Rep. 2016 Feb 17;6:20019. doi: 10.1038/srep20019.
Sci Rep. 2016.
PMID: 26883577
Free PMC article.
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