A hidden threshold in motor neuron gene networks revealed by modulation of miR-218 dose

ND Amin, G Senturk, G Costaguta, S Driscoll… - Neuron, 2021 - cell.com
… , resulted in severe motor neuron synaptic failure and death. This work demonstrates that a
miRNA … Loss of motoneuron-specific microRNA-218 causes systemic neuromuscular failure. …

Motor neuron-derived microRNAs cause astrocyte dysfunction in amyotrophic lateral sclerosis

ML Hoye, MR Regan, LA Jensen, AM Lake, LV Reddy… - Brain, 2018 - academic.oup.com
… We recently demonstrated that microRNA-218 (miR-218) is … This precipitous decline may
suggest a failure of homeostatic … from surrounding glia that causes loss of homeostatic glial …

Human genetics and neuropathology suggest a link between miR-218 and amyotrophic lateral sclerosis pathophysiology

I Reichenstein, C Eitan, S Diaz-Garcia… - Science translational …, 2019 - science.org
… Recent data have shown that microRNA-218 (miR-218) is … miR-218 is essential for perinatal
neuromuscular survival (1, 2), … , developmental loss of miR-218 causes motor neuron death, …

The role of miR-218 in motor neurons

I Reichenstein - 2017 - search.proquest.com
… [67, 68] and because its experimental deficiency causes systemic neuromuscular failure [68].
However, widespread neuronal death in the miR-218 mouse knockout embryos, hindered …

Circulating microRNAs as potential biomarkers and therapeutic targets in spinal muscular atrophy

TH Chen - Therapeutic advances in neurological disorders, 2020 - journals.sagepub.com
… atrophy (SMA), a leading genetic cause of infant death, is a … autosomal recessive
neuromuscular disorder caused by the … profiles of miRNAs may be caused by the failure of …

MotomiRs: miRNAs in motor neuron function and disease

ZCE Hawley, D Campos-Melo… - Frontiers in Molecular …, 2017 - frontiersin.org
… When the action potential has reached the neuromuscular … These experiments showed that
in early development, loss of … Failure to clear Hoxb8 in a spatial and temporal manner leads …

MicroRNA profiling reveals marker of motor neuron disease in ALS models

ML Hoye, ED Koval, AJ Wegener… - Journal of …, 2017 - Soc Neuroscience
… for a miRNA if it failed to be expressed at a … neuromuscular junctions and, ultimately, MN
degeneration (Amin et al., 2015). Although the loss of miR-218 developmentally causes MN …

Multifaceted roles of microRNAs: From motor neuron generation in embryos to degeneration in spinal muscular atrophy

TH Chen, JA Chen - Elife, 2019 - elifesciences.org
10.7554/eLife.50848.001 Two crucial questions in neuroscience are how neurons establish
individual identity in the developing nervous system and why only specific neuron subtypes …

Upregulation of β-catenin due to loss of miR-139 contributes to motor neuron death in amyotrophic lateral sclerosis

S Hawkins, SC Namboori, A Tariq, C Blaker… - Stem Cell Reports, 2022 - cell.com
Loss of motoneuron-specific microRNA-218 causes systemicLoss of motoneuron-specific
microRNA-218 causes systemic neuromuscular failure. …

Spinal muscular atrophy: Selective motor neuron loss and global defect in the assembly of ribonucleoproteins

CE Beattie, SJ Kolb - Brain research, 2018 - Elsevier
… Spinal muscular atrophy is caused by deletions or mutations in the SMN1 gene that result
in … Thus, SMA is an exemplar, selective motor neuron disorder that is caused by defects in …