In a study using a non-toxic substance on mice, Northwestern University researchers have identified the first compound that eliminates the ongoing degeneration of brain neurons in the paralyzing disease known as ALS.

Researchers Hande Ozdinler and Richard Silverman -Northwestern

Amyotrophic lateral sclerosis is a neurodegenerative disease in which upper motor neurons degrade in sufferers, producing a swift and fatal demise.

In addition to ALS, other motor neuron diseases, such as hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS) progress in a similar fashion.

In ALS, movement-initiating nerve cells in the brain (upper motor neurons) and muscle-controlling nerve cells in the spinal cord (lower motor neurons) expire —and, so far, there’s been no medication or treatment for the mind part of ALS, and no medication for HSP and PLS patients.

Even though the upper motor neurons are responsible for the initiation and modulation of motion, and their degeneration is an early event in ALS, so far there has been no treatment choice to increase their health, said senior author Hande Ozdinler, associate professor of neurology at Northwestern University Feinberg School of Medicine.

“ we’ve identified the first compound that boosts the health of upper motor neurons that become diseased. ”

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Ozdinler collaborated with study author Richard B. Silverman, a Northwestern chemistry professor, and published the results in Clinical and Translational Medicine last month on Feb. 23.

The study was initiated following Silverman identified a compound, NU-9, developed in his lab for its ability to reduce protein misfolding in critical cell lines. The compound is not toxic and crosses the blood brain barrier.

I am quite excited to discover if our hypothesis that stabilizing upper motor neurons in mice will translate to humans and NU-9 will provide hope for those inflicted with now untreatable upper motor neuron diseases, Silverman said.

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The NU-9 chemical addresses two of the important elements that cause upper motor neurons to become diseased in ALS: protein misfolding and protein clumping within the cell. Proteins fold in a exceptional way to function ; when they misfold they become poisonous to the neuron. Sometimes proteins aggregate inside the cell and lead to pathology as from the TDP-43 protein pathology. This happens in about 90 percent of all ALS patient brains and is among the most frequent problems in neurodegeneration.

The research team began to investigate whether NU-9 would have the ability to help repair upper motor neurons which become diseased because of increased protein misfolding in ALS. The outcomes in mice were positive. Scientists next performed experiments to reveal how and why the diseased upper motor neurons regained their health.

Restoring neurons to strong health

After administering NU-9, both the mitochondria (the cell’s energy producer ) and the endoplasmic reticulum (the cell ’s protein producer ) began to recover their health and integrity resulting in enhanced neuron health. The upper motor neurons were intact, their cell bodies were larger and the dendrites weren’t riddled with holes.

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They ceased degenerating so much that the diseased neurons became similar to healthy control neurons after 60 days of NU-9 therapy.

Commanders-in-chief of movement

Upper motor neurons are the mind ’s commanders-in-chief of movement. They carry the brain ’s input to spinal cord targets to initiate voluntary movement. The degeneration of these neurons impairs the link from the brain to the spinal cord and contributes to paralysis in patients.

Lower motor neurons have direct connections with the muscle, contracting muscle to execute movement. Therefore, the lower motor neuron activity is in part controlled by the upper motor neurons.

Ozdinler and colleagues will now complete more detailed toxicology and pharmacokinetic studies before initiating a Phase 1 clinical trial.

(Source: Northwestern News Now )

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