Lou Gehrig’s disease, which is considered to be one of the worst and most painful way of dying, is now treatable according to Marcelo Vargas, a postdoctoral fellow in the laboratory of Jeff Johnson, a professor in the School of Pharmacy. The disease is fatal in the sense that the neurons controlling the muscles have already started dying in an untreatable process to bring death within a period of 2-5 years.
Vargas suggested that amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, can be now treated along with prolonging life and slowing down nerve deterioration.
From News-Medical.Net:
The inserted Nrf2 gene was only active in support cells called astrocytes, which promote health among the neurons that actually carry nerve signals, Johnson explains. “We have taken this normal function of producing antioxidants and added to it. It’s like putting the astrocytes on steroids.”
Experiments performed on mouse astrocytes and nerve cells in a dish confirmed the source of the protection, Johnson adds. “We can completely reverse the toxicity of the sick astrocytes. The mutated protein that causes ALS is still there, but Nrf2 makes glutathione that completely blocks it.”
Although the mice that Johnson tested carried the inherited form of ALS, most patients do not have an inherited disease. However, Johnson says the mice are still a good testbed for ALS treatments. “The endpoints that we are blocking, including death of neurons and separation of neurons from the muscle, are seen in all forms of ALS; that’s what makes this so exciting. We are not targeting the mutant protein that causes the disease; we are targeting the astrocytes‘ mechanism that preserves the neurons. The mutant protein is still in all cells of the spinal cord; we are just over-expressing the Nrf2 gene - causing it to make more glutathione - and that provides the protection.”
The study was funded by the Robert Packard Center for ALS Research at Johns Hopkins, the ALS Association and National Institutes of Health grants, and is considered to offer a new approach for treating Lou Gehrig’s disease.
If you would like to make a comment, please fill out the form below.
