June 29, 2022

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Gradual frost disease: Neuronal damage was reversed after 2-month treatment

Gradual frost disease: Neuronal damage was reversed after 2-month treatment

 

Gradual frost disease: Neuronal damage was reversed after 2-month treatment. Gradual frost disease is a more cruel disease than cancer. It will make patients face death consciously.

Amyotrophic lateral sclerosis (ALS), also known as “gradual freezing”, is a rare neurodegenerative disease that attacks nerve cells (neurons) in the human brain and spinal cord, causing muscles to gradually shrink and degenerate .


“Gradual freezing syndrome” is listed as one of the five terminal diseases by the World Health Organization. The cause of the disease is still unknown. Genetics, genetic defects, and environmental factors may all cause motor neuron damage.

Unfortunately, there is still no cure for “gradual freezing”.

The good news is that recently, scientists at Northwestern University in the United States have identified a new compound for the first time, which can treat gradual freezing by reversing the continuous degeneration of upper motor neurons. The lesion of upper motor neuron is the key factor leading to ALS.

 

▌More cruel than cancer: “gradual freezing syndrome”

Motor neurons control the muscle activity during human movement, speech, swallowing and breathing. Once damaged, the muscles atrophy and degenerate, unable to move and speak, swallowing and breathing function will gradually decline, and eventually die of respiratory failure caused by respiratory muscle weakness.

Gradual frost disease: Neuronal damage was reversed after 2-month treatment

In this process, the human body seems to be gradually “frozen”, but the consciousness is very clear, watching oneself die, so it is also regarded as “a disease more cruel than cancer.”

The upper motor neuron is the “commander in chief” of the brain’s movement. In ALS, the motor-initiating nerve cells in the brain (upper motor neuron) and the muscle-controlling nerve cells in the spinal cord (lower motor neuron) die, leading to rapidly deteriorating paralysis and death.


In addition to ALS, upper motor neuron degeneration can also lead to other motor neuron diseases, such as hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS).


Although two drugs, Riluzole and Edaravone, have been approved for “gradual freezing syndrome”, they still focus on symptomatic treatment and cannot be cured.

Moreover, so far, there are no drugs or treatments for the brain nerves of ALS patients, and no drugs for HSP and PLS patients.

 

▌New compound reverses ALS neuron damage

Recently, a study published in “Clinical and Translational Medicine” showed that scientists have identified a compound called NU-9, which can not only reduce the misfolding of proteins in key cell lines, but also pass through the blood. Brain barrier.

Protein misfolding and intracellular protein aggregation are two important factors for motor neuron disease on ALS.

Proteins fold their functions in a unique way, and when they are folded incorrectly, they become toxic to neurons. Sometimes proteins will accumulate in cells like TDP-43 protein pathology and cause pathology.

This condition occurs in the brains of 90% of ALS patients and is one of the most common problems of neurodegeneration.

 

▌2 months later, a miracle happened…


In preclinical trials, researchers gave the new compound NU-9 to test mice.

After taking NU-9, the mitochondria (the energy generator of the cell) and the endoplasmic reticulum (the protein generator of the cell) gradually began to restore their health and integrity, and the health of the neurons was improved.

After 60 days of treatment, the motor neurons in the experimental group of mice became intact, their cell bodies were larger, and there were no perforations in the dendrites, until the diseased neurons returned to levels similar to healthy neurons.

The researchers said: “The NU-9 compound solves two important factors that cause upper motor neurons to develop disease in ALS: protein misfolding and protein aggregation within the cell, which improves the health of brain neurons. But at the beginning of phase 1. Before clinical trials, more detailed toxicology and pharmacokinetic studies need to be completed.”

However, the brilliant performance of this potential novelty in preclinical trials has given us hope for early treatment of patients with “gradual freezing”.

 

 

(source:internet, reference only)


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