User profiles for George Z. Mentis
George MentisColumbia 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
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 (…
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 …
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
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 …
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 …
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
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 …
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…
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 …
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 …
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
Behavioral deficits in neurodegenerative diseases are often attributed to the selective dysfunction
of vulnerable neurons via cell-autonomous mechanisms. Although vulnerable neurons …
of vulnerable neurons via cell-autonomous mechanisms. Although vulnerable neurons …
[HTML][HTML] Molecular correlates of muscle spindle and Golgi tendon organ afferents
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 …
and group Ib Golgi tendon organ (GTO) afferents, but the molecular correlates of these three …