Mouse Motor Cortex Coordinates the Behavioral Response to Unpredicted Sensory Feedback

Heindorf, Matthias; Arber, Silvia; Keller, Georg B.

doi:10.1016/j.neuron.2019.02.042

Cited by 8 publications

(1 citation statement)

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“…The expression of the homeobox gene HB9, a terminal MN transcription factor in the developing spinal cord, was highest at the first time-point measured, day 8, and decreased from then onward (Figure 6I). HB9 expression declines during development (Heindorf et al, 2019;Arber et al, 1999) and longitudinal gene expression studies revealed that it begins to appear 3-5 days after addition of patterning molecules in mouse EB-based ESC-derived MN differentiation protocols (Wichterle et al, 2002;Soundararajan et al, 2006;Amoroso et al, 2013) and 7-14 days in human EB-based iPSC-derived protocols (Amoroso et al, 2013). Our HB9 expression results indicate that in our SCO protocol the specification of MN identity occurred at an early stage.…”

Section: Sma Scos Show Altered Neurodevelopment Which Is Only Partial...mentioning

confidence: 57%

An isogenic human iPSC model unravels neurodevelopmental abnormalities in SMA

Grass

Rosignol

Thomas

et al. 2023

Preprint

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Whether neurodevelopmental defects underlie the selective neuronal death that characterizes neurodegenerative diseases is becoming an intriguing question. To address it, we focused on the motor neuron (MN) disease Spinal Muscular Atrophy (SMA), caused by reduced levels of the ubiquitous protein SMN. Taking advantage of the first isogenic human induced pluripotent stem cell-derived SMA model that we have generated and a spinal cord organoid system, here we report that the relative and temporal expression of early neural progenitor and MN markers is altered in SMA. Furthermore, the corrected isogenic controls only partially reverse these abnormalities. These findings raise the relevant clinical implication that SMN-increasing treatments might not fully amend SMA pathological phenotypes. The approach we have taken demonstrates that the discovery of new disease mechanisms is greatly improved by using human isogenic models. Moreover, our study implies that SMA has a developmental component that might trigger the MN degeneration.

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