Researchers at Upstate make new discovery into cell death and a possible protective mechanism
Principal investigator Xin Jie Chen, PhD, professor of biochemistry and molecular biology, said that his discovery from the decade-long project provides more insight into the physiological impact of mitochondrial dysfunction, which is associated with an increasing number of aging-related neuromuscular degenerative diseases.
“The more research that allows us to gain greater knowledge of how mitochondrial dysfunction induces cell deterioration during aging, the better our chances of developing drugs that delay the onset of cell death and may hold greater hope for drug therapies against these neuromuscular degenerative diseases,” Chen said.
Another finding from the Nature paper is the identification of a network of anti-degenerative genes in yeast that can protect against the mPOS, by detoxifying the dislocated mitochondrial proteins and thereby readjusting cell activity. The anti-degenerative genes are highly conserved in humans, and are mutated in several neuromuscular diseases, including myotonic dystrophy, which affects the muscles, heart, eyes and endocrine system, and spinocerebellar ataxia, a progressive disease that affects movement, speech and vision. The discovery also has implications for amyotrophic lateral sclerosis or Lou Gehrig’s disease, for which there are no known cures and few drugs that are effective in slowing the disease’s progression or reversing its debilitating effect.
“This work is an excellent example of basic scientific research with important implications for human disease,” said Patricia Kane, PhD, professor and chair of the department of biochemistry and molecular biology. “Mitochondria are both the ‘powerhouses of the cell’ and a part of the cell that is very vulnerable to environmental or genetic damage. Dr. Chen’s results provide novel insights into the cellular consequences of mitochondrial damage that could ultimately suggest new treatments for diseases associated with mitochondrial dysfunction.”
Chen and his research team have been supported by grants from the National Institute on Aging (NIH) in the last decade. Chen says building a better understanding of the causes of aging-related neuromuscular degenerative diseases is becoming more imperative as the U.S. population ages. Estimates say that by 2030 the proportion of the U.S. population over 65 will double or one in every five Americans, making it likely that the number of individuals with these disorders will likely increase. Studying the basic principles of aging-related cell death is critical for developing anti-degenerative interventions and promoting healthy aging, researchers say.
The lead author of the Nature paper is Xiaowen Wang, a research scientist at Upstate Medical University, also in the Department of Biochemistry and Molecular Biology.