• Amyotrophic Lateral Sclerosis (ALS)

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting adults characterized by the loss of spinal and corticospinal motor neurons. Patients first present with tremors and weakness but rapidly progress to paralysis and eventually death.

    Recently, it has become apparent that multiple cell types are important for disease progression in ALS. We have discovered that the V2a class of excitatory interneurons degenerate early in a mouse model of ALS and we are now investigating whether interneuron degeneration contributes to disease pathogenesis or is merely a consequence of disease.

    These studies will have important implications for understanding the role of neural circuits in other neurodegenerative diseases, such as: Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, and spinal muscular atrophy.

    Microglial cell enveloping a V2a neuron in a mouse model of ALS. (Red= Iba-1, green= CFP) Microglial cell enveloping a V2a neuron in a mouse model of ALS. (Red= Iba-1, green= CFP)
    V2a interneuron expressing mis-folded SOD1 protein in the SOD1G93A mouse model of ALS. (red= mis-folded SOD1, green= CFP) V2a interneuron expressing mis-folded SOD1 protein in the SOD1G93A mouse model of ALS. (red= mis-folded SOD1, green= CFP)
    Confocal image of motor neurons in the ventral horn of the spinal cord (red= IRF3, blue= DAPI) Confocal image of motor neurons in the ventral horn of the spinal cord (red= IRF3, blue= DAPI)