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Friday, June 21, 2024
NIH-funded research sheds light on disease use, points to specific targets
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In a small study, researchers discovered how the death of neurons in amyotrophic lateral sclerosis (ALS) develops. Results, published in Natural Parenting, provide information on the cause of ALS and may lead to new ways to stop the progression of the disease. The study was funded by the National Institutes of Health (NIH).
ALS is a progressive disease that attacks motor neurons, nerve cells in the brain and the vessels that control muscles, leading to muscle weakness, paralysis, and eventually death. Most cases of ALS are progressive and occur in people with no family history or other obvious problems.
By analyzing the genetic profiles of thousands of neurons from postmortem brains from people diagnosed with ALS and from healthy donors, the researchers identified high levels of risk genes for ALS and frontotemporal dementia (FTD). The genes that were most prominent in Betz cells, a type of motor nerve, express the signal It’s YOUR1. In people with ALS, it is associated with disturbances in other neurons, disrupting their ability to build, transport, and break down proteins. Genetics—including SOD1, KIF5Aand CHCHD10-one of the factors commonly associated with ALS/FTD.
Additional studies have shown that these changes may be related to the toxic accumulation of the protein TDP-43, a defining feature of ALS and some cases of FTD. Therefore, the high level of ALS risk genes in a type of cells can trigger a destructive reaction that leads to a lot of loss.
Betz cell degeneration is a hallmark of ALS and is thought to occur early when symptoms first appear. Understanding how these and other cells may be affected in ALS could lead to new treatments that slow and stop the disease’s progression.
The team also investigated how glial cells are affected in ALS. Glia are the supporting cells that normally keep neurons healthy, but in ALS they can become damaged and destroy neurons, often accelerating their decline. The researchers analyzed the genetic information from the two types of glial cells and found genes associated with infection and inflammation. More research is needed to determine whether glial cell dysfunction is a consequence or a cause of neuron damage in ALS.
Together, the results improve our understanding of why certain neurons are more affected in ALS and identify new treatment strategies.
The study was supported by the National Institute of Neurological Disorders and Stroke (NINDS) (K08NS104270) and the National Institute on Aging (NIA) (P30AG062421). The single-cell sequencing and other resources were provided by the NIA-funded Massachusetts Alzheimer’s Disease Research Center, one of 35 centers located across the United States.
WHO
Amelie Gubitz, Ph.D., program director, NINDS, is available to discuss the research. To schedule an interview, please contact NINDSPressTeam@ninds.nih.gov.
Article
Limone, F., et al. “Single nucleus accumbens reveals enrichment of effector expression in extratelencephalic neurons sensitive to damage in ALS.” Natural Parenting. June 21, 2024. DOI: 10.1038/s43587-024-00640-0.
NINDS is the nation’s leading funder of brain and body research. The mission of NINDS is to find basic knowledge about the brain and how the body works and to use that knowledge to reduce the burden of neurological diseases.
About the National Institute on Aging (NIA): The NIA leads the US government’s effort to conduct and support research on aging and the health and well-being of older adults. Learn more about cognitive changes and age-related neurodegenerative diseases through the NIA’s Alzheimer’s and related Dementias Education and Referral (ADEAR) Center website. Visit the NIA’s main website for information on a wide range of issues, in English and Spanish, and stay connected.
About the National Institutes of Health (NIH):NIH, the nation’s national medical research agency, consists of 27 Institutes and Centers and is part of the US Department of Health and Human Services. The NIH is the primary federal agency that conducts and supports clinical, clinical, and translational medical research, and investigates the causes, treatments, and cures for common and rare diseases. For more information about NIH and its programs, visit www.nih.gov.
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