Motor Neuron Center News Search

Showing 11 - 20 of 21 results.

Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy

The hallmark of spinal muscular atrophy (SMA), an inherited disease caused by ubiquitous deficiency in the SMN protein, is the selective degeneration of subsets of spinal motor neurons.

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Research
Research Unveils a Developmental Splicing Program Controlling Neuronal Maturation and Excitability

In a new study published in Neuron, Professors Chaolin Zhang and Hynek Wichterle determined that loss of Rbfox genes results in an “embryonic like” splicing program.
The Regulatory Machinery of Neurodegeneration in In Vitro Models of Amyotrophic Lateral Sclerosis

The discovery of key mediators of neurodegenerative phenotypes, especially prior to neurons becoming committed to die, has proven both complex and inefficient.

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Research
The ALS Association Program at Columbia University Named a Certified Treatment Center of Excellence

The ALS Association Program at Columbia University has successfully achieved certification as a Certified Treatment Center of Excellence (CTCE) through The ALS Association.
A Pathogenic S250F Missense Mutation Results in a Mouse Model of Mild Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency

Homozygous mutations in the aromatic l-amino acid decarboxylase (AADC) gene result in a severe depletion of its namesake protein

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Research
Motor Neuronal Repletion of the NMJ Organizer, Agrin, Modulates the Severity of the Spinal Muscular Atrophy Disease Phenotype in Model Mice

Spinal muscular atrophy (SMA) is a common and often fatal neuromuscular disorder caused by low levels of the Survival Motor Neuron (SMN) protein.

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Research
Dysregulation of Mdm2 and Mdm4 Alternative Splicing Underlies Motor Neuron Death in Spinal Muscular Atrophy

Ubiquitous deficiency in the survival motor neuron (SMN) protein causes death of motor neurons.

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Research
New Insights on How the Reprogramming Factor LIN28 Regulates Its Targets

By Melanie A. Farmer

A new study led by Chaolin Zhang, PhD, assistant professor of systems biology, published today as the cover story of Molecular Cell, sheds light on a critical RNA-binding protein.

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Research
FUS Affects Circular RNA Expression in Murine Embryonic Stem Cell-Derived Motor Neurons

The RNA-binding protein FUS participates in several RNA biosynthetic processes and has been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia.

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Research
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.

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Research

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