Thursday, September 7, 2017

RNA biology of disease-associated microsatellite repeat expansions

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Kushal J. Rohilla and Keith T. Gagnon, Acta Neuropathologica 20175:63, doi:10.1186/s40478-017-0468-y This review focuses on the potential ...
Wednesday, September 6, 2017

A Defective mRNA Cleavage and Polyadenylation Complex Facilitates Expansions of Transcribed (GAA)n Repeats Associated with Friedreich’s Ataxia

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Ryan J. McGinty, Franco Puleo, Anna Y. Aksenova, Julia A. Hisey, Alexander A. Shishkin, Erika L. Pearson, Eric T. Wang, David E. Housman, Cl...
Tuesday, September 5, 2017

Engineered Axonal Tracts as “Living Electrodes” for Synaptic-Based Modulation of Neural Circuitry

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M. D. Serruya, J. P. Harris, D. O. Adewole, L. A. Struzyna, J. C. Burrell, A. D. Nemes, D. Petrov, R. H. Kraft, H. I. Chen, J. A. Wolf, D. K...
Thursday, August 31, 2017

Biogenesis and functions of mammalian iron-sulfur proteins in the regulation of iron homeostasis and pivotal metabolic pathways.

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Tracey A. Rouault and Nunziata Maio; The Journal of Biological Chemistry 292, 12744-12753. doi: 10.1074/jbc.R117.789537 Fe-S cofactors are...
Wednesday, August 30, 2017

Peptide SS-31 upregulates frataxin expression and improves the quality of mitochondria: implications in the treatment of Friedreich ataxia

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Hongting Zhao, Huihui Li, Shuangying Hao, Jiping Chen, Jing Wu, Chuanhui Song, Meng Zhang, Tong Qiao & Kuanyu Li; Scientific Reports 7, ...
Sunday, August 27, 2017

Overexpression of Drosophila frataxin triggers cell death in an iron-dependent manner

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Oliver Edenharter, Janik Clement, Stephan Schneuwly & Juan A. Navarro, Journal of Neurogenetics Vol. 0 , Iss. 0,0; doi:10.1080/01677063....
Saturday, August 26, 2017

Are Astrocytes the Predominant Cell Type for Activation of Nrf2 in Aging and Neurodegeneration?

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Jeffrey R. Liddell, Antioxidants 2017, 6(3), 65; doi:10.3390/antiox6030065 This review describes the evidence for Nrf2 activation in each ...
Wednesday, August 23, 2017

Jupiter Orphan Therapeutics Receives Orphan Drug Designation for its Trans-Resveratrol Product JOTROL for Treatment of Friedreich's Ataxia

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JUPITER, Fla., Aug. 22, 2017 /PRNewswire/ -- Jupiter Orphan Therapeutics, Inc. ("JOT"), Jupiter, FL, today announced that it has r...
Tuesday, August 22, 2017

Assessment of glutathione/glutathione disulphide ratio and S-glutathionylated proteins in human blood, solid tissues, and cultured cells

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Daniela Giustarini, Graziano Colombo, Maria Lisa Garavaglia, Emanuela Astori, Nicola Marcello Portinaro, Francesco Reggiani, Salvatore Badal...
Monday, August 21, 2017

European Reference networks for rare diseases: what is the conceptual framework?

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Véronique Héon-Klin, Orphanet Journal of Rare Diseases 201712:137 doi:10.1186/s13023-017-0676-3 In February 2017, 24 European Reference Ne...