Inhibition of inflammation with celastrol fails to improve muscle function in dysferlin-deficient A/J mice

Autor: Dillingham, BC, Klimek, MEB, Gernapudi, R, Rayavarapu, S, Gallardo, E, Van der Meulen, JH, Jordan, S, Ampong, B, Gordish-Dressman, H, Spurney, CF, Nagaraju, K

Publikováno v: JOURNAL OF THE NEUROLOGICAL SCIENCES
r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
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The dysferlin-deficient A/J mouse strain represents a homologous model for limb-girdle muscular dystrophy 2B. We evaluated the disease phenotype in 10 month old A/J mice compared to two dysferlin-sufficient, C57BL/6 and ALOlaHsd, mouse lines to deter

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Inhibition of inflammation with celastrol fails to improve muscle function in dysferlin-deficient A/J mice.

Autor: Dillingham BC; Research Center for Genetic Medicine, Children's National Medical Center, 111 Michigan Ave NW, Washington, D.C., USA., Benny Klimek ME; Research Center for Genetic Medicine, Children's National Medical Center, 111 Michigan Ave NW, Washington, D.C., USA., Gernapudi R; Research Center for Genetic Medicine, Children's National Medical Center, 111 Michigan Ave NW, Washington, D.C., USA., Rayavarapu S; Research Center for Genetic Medicine, Children's National Medical Center, 111 Michigan Ave NW, Washington, D.C., USA., Gallardo E; Institut de Recerca Hospital de la Santa Creu i Sant Pau. U.A.B.C./Pare Claret, 167 08025 Barcelona, Spain., Van der Meulen JH; Institut de Recerca Hospital de la Santa Creu i Sant Pau. U.A.B.C./Pare Claret, 167 08025 Barcelona, Spain., Jordan S; Institut de Recerca Hospital de la Santa Creu i Sant Pau. U.A.B.C./Pare Claret, 167 08025 Barcelona, Spain., Ampong B; Institut de Recerca Hospital de la Santa Creu i Sant Pau. U.A.B.C./Pare Claret, 167 08025 Barcelona, Spain., Gordish-Dressman H; Institut de Recerca Hospital de la Santa Creu i Sant Pau. U.A.B.C./Pare Claret, 167 08025 Barcelona, Spain., Spurney CF; Institut de Recerca Hospital de la Santa Creu i Sant Pau. U.A.B.C./Pare Claret, 167 08025 Barcelona, Spain., Nagaraju K; Research Center for Genetic Medicine, Children's National Medical Center, 111 Michigan Ave NW, Washington, D.C., USA; Department of Integrative Systems Biology, Institute for Biomedical Sciences, The George Washington University, 2300 Eye Street, N.W., Ross 605, Washington, D.C., USA. Electronic address: knagaraju@childrensnational.org.

Publikováno v: Journal of the neurological sciences [J Neurol Sci] 2015 Sep 15; Vol. 356 (1-2), pp. 157-62. Date of Electronic Publication: 2015 Jun 24.