Christian M. Simon

A standardized framework resolves ambiguity in motor neuron loss across neurodegenerative diseases

This paper develops a standardized framework to accurately quantify motor neuron loss across neurodegenerative diseases by addressing variability in sampling and marker specificity.

Incomplete cerebellar circuit restoration limits functional recovery following SMN therapy in severe spinal muscular atrophy

The paper identifies cerebellar pathology as a conserved yet distinct feature in severe SMA, showing divergent therapeutic outcomes with SMN-restoring therapies.

A p53-ΔNp73 signaling axis drives selective motor neuron degeneration in spinal muscular atrophy

This study identifies a p53-ΔNp73 signaling axis that drives motor neuron degeneration in SMA, revealing a context-dependent switch in p53 family signaling that promotes neurodegeneration.

Peripherally derived LGI1-reactive monoclonal antibodies cause epileptic seizures in vivo

This study shows that LGI1-reactive monoclonal antibodies cause seizures in rats, with molecular and electrophysiological evidence of epileptogenesis.

SMN controls neuromuscular junction integrity through U7 snRNP

This study identifies a role for SMN in maintaining neuromuscular junction integrity through U7 snRNP function in SMA mice.

Stasimon contributes to the loss of sensory synapses and motor neuron death in a mouse model of spinal muscular atrophy

This study shows that Stasimon overexpression in SMA mice improves motor function by preventing synaptic loss and motor neuron death through mechanisms involving p53 regulation.