User profiles for George Z. Mentis

George Mentis

Columbia University
Verified email at mentislab.org
Cited by 4163

[PDF][PDF] Early functional impairment of sensory-motor connectivity in a mouse model of spinal muscular atrophy

GZ Mentis, D Blivis, W Liu, E Drobac, ME Crowder… - Neuron, 2011 - cell.com
To define alterations of neuronal connectivity that occur during motor neuron degeneration,
we characterized the function and structure of spinal circuitry in spinal muscular atrophy (…

[HTML][HTML] ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

…, M Mendelsohn, A Nemes, JC Tapia, GZ Mentis… - Nature …, 2016 - nature.com
Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most
aggressive, juvenile-onset forms of the disease. FUS loss-of-function and toxic gain-of-function …

[HTML][HTML] An SMN-dependent U12 splicing event essential for motor circuit function

…, L Saieva, ES Beck, LT Hao, DK Li, W Jiao, GZ Mentis… - Cell, 2012 - cell.com
Spinal muscular atrophy (SMA) is a motor neuron disease caused by deficiency of the
ubiquitous survival motor neuron (SMN) protein. To define the mechanisms of selective neuronal …

[PDF][PDF] Spinal inhibitory interneuron diversity delineates variant motor microcircuits

…, E Famojure, C Diaz, FJ Alvarez, GZ Mentis… - Cell, 2016 - cell.com
Animals generate movement by engaging spinal circuits that direct precise sequences of
muscle contraction, but the identity and organizational logic of local interneurons that lie at the …

Gain of toxic function by long-term AAV9-mediated SMN overexpression in the sensorimotor circuit

…, LS Shihabuddin, C Zhang, GZ Mentis… - Nature …, 2021 - nature.com
The neurodegenerative disease spinal muscular atrophy (SMA) is caused by deficiency in
the survival motor neuron (SMN) protein. Currently approved SMA treatments aim to restore …

Noncholinergic excitatory actions of motoneurons in the neonatal mammalian spinal cord

GZ Mentis, FJ Alvarez, A Bonnot… - Proceedings of the …, 2005 - National Acad Sciences
Mammalian spinal motoneurons are considered to be output elements of the spinal cord that
generate exclusively cholinergic actions on Renshaw cells, their intraspinal synaptic targets…

[PDF][PDF] The classical complement pathway mediates microglia-dependent remodeling of spinal motor circuits during development and in SMA

…, TA Yednock, BA Barres, GZ Mentis - Cell reports, 2019 - cell.com
Movement is an essential behavior requiring the assembly and refinement of spinal motor
circuits. However, the mechanisms responsible for circuit refinement and synapse …

Impaired prenatal motor axon development necessitates early therapeutic intervention in severe SMA

…, NA Naryshkin, TO Crawford, GZ Mentis… - Science translational …, 2021 - science.org
Gene replacement and pre-mRNA splicing modifier therapies represent breakthrough gene
targeting treatments for the neuromuscular disease spinal muscular atrophy (SMA), but …

Reduced sensory synaptic excitation impairs motor neuron function via Kv2. 1 in spinal muscular atrophy

…, A Vukojicic, E Drobac, X Wang, GZ Mentis - Nature …, 2017 - nature.com
Behavioral deficits in neurodegenerative diseases are often attributed to the selective dysfunction
of vulnerable neurons via cell-autonomous mechanisms. Although vulnerable neurons …

[HTML][HTML] Molecular correlates of muscle spindle and Golgi tendon organ afferents

KM Oliver, DM Florez-Paz, TC Badea, GZ Mentis… - Nature …, 2021 - nature.com
Proprioceptive feedback mainly derives from groups Ia and II muscle spindle (MS) afferents
and group Ib Golgi tendon organ (GTO) afferents, but the molecular correlates of these three …