George Mentis received pursued B.Sc in Physiology and M.Sc in Neurological Science from University College London (UCL). He completed his PhD in Neuroscience from Imperial College London, UK. George Mentis worked initially as a Postdoctoral fellow and subsequently as a Staff Scientist at NINDS in the NIH, Bethesda Maryland. 

Dr. Mentis’s lab is currently concentrating on mouse models of SMA that closely mimic severe forms of the disease in infants. Mutant SMN-deficient mice exhibit severe motor abnormalities at birth and die within two weeks. To date, the majority of SMA studies have focused on molecular aspects of motor neuron degeneration and on changes at the neuromuscular junction. Little is known however, about the pathophysiology of the disease as it relates to spinal cord circuitry and motor neuron activity. Dr. Mentis and his lab members are currently studying the effects of the SMN deficiency in specific neuronal populations.

Research Interests

• Motor Systems

• Neural Degeneration and Repair

• Sensory Physiology

• Synapses and Circuits

Representative Publications:

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

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

Reduced Sensory Synaptic Excitation Impairs Motor Neuron Function via Kv2.1 in Spinal Muscular Atrophy

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