Yet, it remains poorly understood how early in the disease cortical spinal neurons (CSNs) show these alterations, or whether CSN/CST pathology resembles the abnormalities observed in other tissues affected by FXN loss. To address these questions, we examined CSN driven motor behaviors and pathology in the YG8JR FRDA mouse model. We find that FRDA mice show impaired motor skills, exhibit significant reductions in CSN functional output, and, among other pathological changes, show abnormal mitochondrial distributions in CSN neurons and CST axonal tracts. Moreover, some of these alterations were observed as early as two months of age, suggesting that CSN/CST pathology may be an earlier event in FRDA disease than previously appreciated. These studies warrant a detailed mechanistic understanding of how FXN loss impacts CSN health and functionality.
Tuesday, July 30, 2024
Anatomical and functional analysis of the corticospinal tract in an FRDA mouse model
Anatomical and functional analysis of the corticospinal tract in an FRDA mouse model. Misa Nishiyama, John Kalambogias, Fumiyasu Imai, Emily Yang, Sonia Lang, Joriene C de Nooij, Yutaka Yoshida
bioRxiv 2024.06.28.601178; doi:10.1101/2024.06.28.601178
