Principal Investigator, Linus Pauling Institute
University Distinguished Professor Emeritus, Department of Biochemistry and Biophysics
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Research Interests
My research focuses on unraveling the causes of Lou Gehrig’s Disease, commonly known as amyotrophic lateral sclerosis (ALS). ALS is a devastating disease characterized by the unexplained and progressive death of motor neurons that control voluntary muscle movement. As a result, victims gradually become paralyzed, with survival ranging from months to several years.
In 1993, dominant mutations in the antioxidant enzyme copper/zinc superoxide dismutase were identified as the cause of ALS in approximately 2-5% of cases. Subsequently, in the late 1990s, we developed a theory to explain how superoxide dismutase could lead to ALS, which sparked controversy due to seemingly contradictory findings.
In light of the advancements in new technologies, we have forged international collaborations to address these controversies with innovative approaches that pave the way for improved treatments of the disease.
Education
- B.A., Molecular Biology, University of Colorado (1975)
- M.A., Molecular Biology, University of Colorado (1977)
- CPT, US ARMY Medical Services Command (1975-1991)
- Ph.D. Botany, Duke University (1984)
- Post Doc, Pulmonary Medicine, Duke University (1985)
Featured Publications
Visioli F, Ingram A, Beckman JS, Magnusson KR, Hagen TM. (2022) Strategies to protect against age-related mitochondrial decay: Do natural products and their derivatives help? Free Radic Biol Med. 178:330-346. doi: 10.1016/j.freeradbiomed.2021.12.008.
Trias E, Beilby PR, Kovacs M, Ibarburu S, Varela V, Barreto-Núñez R, Bradford SC, Beckman JS, Barbeito L. (2019) Emergence of microglia bearing senescence markers during paralysis progression in a rat model of inherited ALS. Front Aging Neurosci. 11:42. doi: 10.3389/fnagi.2019.00042.
Trias E, Ibarburu S, Barreto-Núñez R, Babdor J, Maciel TT, Guillo M, Gros L, Dubreuil P, Díaz-Amarilla P, Cassina P, Martínez-Palma L, Moura IC, Beckman JS, Hermine O, Barbeito L. (2016) Post-paralysis tyrosine kinase inhibition with masitinib abrogates neuroinflammation and slows disease progression in inherited amyotrophic lateral sclerosis. J Neuroinflammation. 13:177. doi: 10.1186/s12974-016-0620-9.
Williams JR, Trias E, Beilby PR, Lopez NI, Labut EM, Bradford CS, Roberts BR, McAllum EJ, Crouch PJ, Rhoads RW, Pereira C, Son M, Elliot JL, Franco MC, Estévez AG, Barbeito L, Beckman JS. (2016) Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SOD(G93A) mice co-expressing the Copper-Chaperone-for-SOD. Neurobiol Dis. 89:1-9. doi: 10.1016/j.nbd.2016.01.020.
Roberts BR, Lim NKH, McAllum EJ, Donnelly PS, Hare DJ, Doble PA, Turner BJ, Price KA, Lim SC, Paterson BM, Hickey JL, Rhoads TW, Williams JR, Kanninen KM, Hung LW, Liddell JR, Grubman A, Monty JF, Llanos RM, Kramer DR, Mercer JFB, Bush AI, Masters CL, Duce JA, Li QX, Beckman JS, Barnham KJ, White AR, Crouch PJ. (2014) Oral treatment with CuII(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci. 34(23):8021-8031. doi: 10.1523/JNEUROSCI.4196-13.2014.
Trias E, Díaz-Amarilla P, Olivera-Bravo S, Isasi E, Drechsel DA, Lopez N, Bradford CS, Ireton KE, Beckman JS, Barbeito L. (2013) Phenotypic transition of microglia into astrocyte-like cells associated with disease onset in a model of inherited ALS. Front Cell Neurosci. 7:274. doi: 10.3389/fncel.2013.00274.
Franco MC, Ye Y, Refakis CA, Feldman JL, Stokes AL, Basso M, Melero-Fernández de Mera RM, Sparrow NA, Calingasan NY, Kiaei M, Rhoads TW, Ma TC, Grumet M, Barnes S, Beal MF, Beckman JS, Mehl R, Estévez AG (2013) Nitration of Hsp90 induces cell death. Proc Natl Acad Sci USA. 110(12):E1102-1011. doi: 10.1073/pnas.1215177110.