George Z. Mentis

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.

C3 and CD47 mediate sensory-motor circuit refinement during spinal cord development

This study identifies C3 and CD47 as dual mechanisms refining spinal sensory-motor circuits by eliminating inappropriate synapses during development.

A mouse model of CHCHD10 p.R15L familial ALS presents mild, age-related motor neuron degeneration without protein instability or mitochondrial dysfunction

Synaptic imbalance and increased inhibition impair motor function in SMA

This study shows that unbalanced excitation-inhibition neurotransmission in SMA mice impairs motor function, and reducing inhibition improves neuronal function and behavior.

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.

Molecular development of muscle spindle and Golgi tendon organ sensory afferents revealed by single proprioceptor transcriptome analysis

The paper identifies molecularly distinct proprioceptor subtypes in muscle spindles and Golgi tendon organs using single-cell RNA sequencing and validates their functional roles.

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.