The model simulations revealed that the feedback delay, but not the feedback gain was affected in FA patients. They also showed that the descending control signal was scaled in time and amplitude and co-contraction was smaller in FA patients. Thus, our study explains how the motor deficits of FA patients result from pathological alterations of both top-down and feedback control.
Wednesday, March 16, 2022
Control of arm movements in Friedreich's ataxia patients: role of sensory feedback
Zhang L, Straube A, Eggert T. ; Exp Brain Res. 2022 Mar 14. doi: 10.1007/s00221-022-06343-5. Epub ahead of print. PMID: 35286422.
Difficulties translating antisense-mediated activation of Frataxin expression from cell culture to mice
Audrius Kilikevicius, Jun Wang, Xiulong Shen, Frank Rigo, Thahza P. Prakash, Marek Napierala & David R. Corey (2022); RNA Biology, 19:1, 364-372, DOI: 10.1080/15476286.2022.2043650
In this study, we investigate the potential for oligonucleotides to increase frataxin expression in a mouse model for FA. After confirming successful in vivo delivery of oligonucleotides using a benchmark gapmer targeting the nuclear noncoding RNA Malat1, we tested anti-FXN oligonucleotides designed to function by various mechanisms. None of these strategies yielded enhanced expression of FXN in the model mice. Our inability to translate activation of FXN expression from cell culture to mice may be due to inadequate potency of our compounds or differences in the molecular mechanisms governing FXN gene repression and activation in FA model mice.
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