NEW YORK, Jun. 15, 2015 /PRNewswire-iReach/ -- Stuart Millheiser founded the charity organization ALS Guardian Angels. Millheiser and his charity support patients with amyotrophic lateral sclerosis by providing medical equipment, emotional support and outspoken advocacy for ALS research.
According to Stu Millheiser, amyotrophic lateral sclerosis, or "motor neuron disease" in other countries, is a chronically debilitating neurodegenerative disorder that leads to paralysis. ALS causes the destruction of motor neurons in the spinal cord and brain.
Now, scientists from the University of Michigan and the Gladstone Institutes have identified a specific cellular process that can help researchers focus their attention on finding a drug that could possibly treat ALS and frontotemporal dementia, a related disorder.
According to the team of researchers, one of the main commonalities of ALS is the accumulation of TDP43, a protein that, in excess, can cause toxicity at the cellular level. For the first time, scientists have been able to link neurodegenerative disease to this natural system of monitoring.
During the study, researchers were able to identify another protein, hUPF1, which plays a crucial role in stabilizing TDP43 and, thereby, preventing the death of cells. TDP43 is an important protein for cellular health, but too little or too much of it can destroy cells.
In at least 90 percent of ALS cases, patients exhibit TDP43-based pathology. This fact makes it imperative that scientists develop a treatment that can maintain optimal levels of this protein. The findings of this study revealed that increasing hUPF1 levels genetically lengthens the lifespan of affected neurons, with a survival extension rate of 60 percent.
Furthermore, an additional discovery shows that hUPF1 acts through nonsense-mediated decay, a system of cellular surveillance, to stabilize levels of TDP43 and enhance survival times of neurons. According to the researchers, cells have a unique way of maintaining homeostasis and protecting against faulty proteins, and this new understanding paves the way for potential treatments.
This is the first time anyone has identified the link between neurodegenerative disorders and the body's natural monitoring system. This discovery provides guidance for future research into ALS treatments.
Using these findings, scientists hope to develop a drug that can specifically target nonsense-mediated decay. The results of this study offer great hope that manipulating this surveillance system with hUPF1, or through similar proteins that act in the same way, can protect neurons and influence unstable levels of TDP43 and their destructive consequences.
Stuart Millheiser is the founder of ALS Guardian Angels. Millheiser founded ALS Guardian Angels in 2007 to help patients that are suffering from ALS, formerly known as Lou Gehrig's disease. Millheiser has helped hundreds of people with ALS by providing everything from medical equipment to support for basic living expenses.
Millheiser has been referred to as "a true angel on earth" for his charitable work. He feels a sense of accomplishment by helping those in need. After the viral spreading of the ice bucket challenge during the summer of 2014, Millheiser is hopeful that more people will continue to become aware of ALS and support this truly important cause.
Media Contact:Stuart Millheiser, ALS Guardian Angels, 949-488-9894, info@alsguardianangels.com
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SOURCE ALS Guardian Angels
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