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Researchers from University of California Report on Findings in Amyotrophic Lateral Sclerosis

Science Letter


By a News Reporter-Staff News Editor at Science Letter -- Researchers detail new data in Nervous System Diseases and Conditions. According to news originating from La Jolla, California, by NewsRx correspondents, research stated, "Humans with ALS and transgenic rodents expressing ALS-associated superoxide dismutase (SOD1) mutations develop spontaneous blood-spinal cord barrier (BSCB) breakdown, causing microvascular spinal-cord lesions. The role of BSCB breakdown in ALS disease pathogenesis in humans and mice remains, however, unclear, although chronic blood-brain barrier opening has been shown to facilitate accumulation of toxic blood-derived products in the central nervous system, resulting in secondary neurodegenerative changes."

Our news journalists obtained a quote from the research from the University of California, "By repairing the BSCB and/or removing the BSCB-derived injurious stimuli, we now identify that accumulation of blood-derived neurotoxic hemoglobin and iron in the spinal cord leads to early motor-neuron degeneration in SOD1(G93A) mice at least in part through iron-dependent oxidant stress. Using spontaneous or warfarin-accelerated microvascular lesions, motor-neuron dysfunction and injury were found to be proportional to the degree of BSCB disruption at early disease stages in SOD1(G93A) mice. Early treatment with an activated protein C analog restored BSCB integrity that developed from spontaneous or warfarin-accelerated microvascular lesions in SOD1(G93A) mice and eliminated neurotoxic hemoglobin and iron deposits. Restoration of BSCB integrity delayed onset of motor-neuron impairment and degeneration. Early chelation of blood-derived iron and antioxidant treatment mitigated early motor-neuronal injury."

According to the news editors, the research concluded: "Our data suggest that BSCB breakdown contributes to early motor-neuron degeneration in ALS mice and that restoring BSCB integrity during an early disease phase retards the disease process."

For more information on this research see: Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice. Proceedings of the National Academy of Sciences of the United States of America, 2014;111(11):E1035-E1042. Proceedings of the National Academy of Sciences of the United States of America can be contacted at: Natl Acad Sciences, 2101 Constitution Ave NW, Washington, DC 20418, USA. (National Academy of Sciences - www.nasonline.org/; Proceedings of the National Academy of Sciences of the United States of America - www.nasonline.org/publications/pnas/)

The news correspondents report that additional information may be obtained from E.A. Winkler, University of California, Ludwig Inst Canc Res, Dept. of Cellular & Mol Med, La Jolla, CA 92093, United States. Additional authors for this research include J.D. Sengillo, A.P. Sagare, Z. Zhao, Q.Y. Ma, E. Zuniga, Y.M. Wang, Z.H. Zhong, J.S. Sullivan, J.H. Griffin, D.W. Cleveland and B.V. Zlokovic (see also Nervous System Diseases and Conditions).

Keywords for this news article include: La Jolla, California, Spinal Cord, United States, North and Central America, Amyotrophic Lateral Sclerosis, Central Nervous System Diseases, Nervous System Diseases and Conditions

Our reports deliver fact-based news of research and discoveries from around the world. Copyright 2014, NewsRx LLC

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