Researchers Identify Novel Protein to Treat In Lou Gehrig’s Disease

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Researchers from Case Western Reserve University suggest that a supplement of protein found in the spinal cord could treat muscle disorders.

Amyotrophic lateral sclerosis (ALS), also known Lou Gehrig’s disease, is a progressive disorder that weakens muscles and impacts physical functions such as speaking, eating, moving, or breathing. The cellular mechanism behind ALS is similar to certain types of dementia. According to Centers for Disease Control and Prevention (CDC), ALS prevalence was at 5.0 cases per 100,000 persons in 2014. Although a medication called riluzole may extend life by about two to three months, no cure for the disease is known. Now, researchers from Case Western Reserve University School of Medicine suggest that a supplement of a protein called mitofusion 2 (Mfn2) found in the spinal cord could prevent symptoms of ALS. A mouse model showed that Mfn2 prevented nerve degeneration, muscle atrophy, and paralysis. Furthermore, supplementing the protein could be a novel therapeutic approach for ALS as Mfn2 is often depleted during the disease. The research led by Xinglong Wang, PhD, associate professor of pathology at Case Western Reserve University School of Medicine was published in Cell Metabolism on July 12, 2018.

The researchers genetically engineered the ALS mice to have increased Mfn2 levels only in nerve cells that extend from the spinal cord and connect to muscle fibers. It was observed that in later stages of the disease, mice with high Mfn2 levels in these nerves were a healthy weight. Furthermore, no sign of muscle atrophy, gait abnormalities, or reduced grip strength was evident in the mice and the mice who underwent heavy sciatic nerve damage benefited from elevated Mfn2 levels. The nerve cells collected from the mice revealed that mitochondria contained Mfn2. This mitochondria delivers the nutrients to the point where nerve cells and muscle fibers meet, which in turn preserves the synapses between nerve and muscle cells and prevent muscle atrophy. The findings are expected to help develop effective therapeutic approach to treat a variety of muscular disorders, nerve injuries, and various major neurodegenerative diseases associated with synaptic loss.

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