Wednesday, October 12, 2022

SARS-CoV-2 in patients with Friedreich ataxia

Megan M. Shen, Layne N. Rodden, Kellie McIntyre, Adriana Arias, Victoria Profeta, Kimberly Schadt & David R. Lynch; J Neurol (2022). doi:10.1007/s00415-022-11419-x

On a broader scale, the pandemic’s effects on this cohort are likely more prominent not from infection itself but rather disruptions to physical activity and therapy services. Anecdotally, a substantial proportion of patients have perceived significant declines in global health resulting from these disruptions, consistent with previous reports [1]. Future investigation with an expanded cohort and updated vaccination data as novel SARS-CoV-2 variants emerge will continue to inform adaptations in care.

A promising mouse model for Friedreich Ataxia progressing like human patients

Catherine GĂ©rard, Annabelle Fortin Archambault, Camille Bouchard, Jacques P. Tremblay; Behavioural Brain Research, Volume 436, 2023, 114107, doi:10.1016/j.bbr.2022.114107. 

Jackson Laboratories Inc. developed a new mouse model that has 800 GAA repeats. We demonstrate here that these mice accurately reflect the human disease with a progressive neuromuscular degeneration highlighted by the two beam tests and the beginning of heart hypertrophy at 26 weeks. YG8-800 mice are thus currently a promising mouse model for FRDA.