A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.

Autor: Robert C C Mercer, Nathalie Daude, Lyudmyla Dorosh, Ze-Lin Fu, Charles E Mays, Hristina Gapeshina, Serene L Wohlgemuth, Claudia Y Acevedo-Morantes, Jing Yang, Neil R Cashman, Michael B Coulthart, Dawn M Pearson, Jeffrey T Joseph, Holger Wille, Jiri G Safar, Gerard H Jansen, Maria Stepanova, Brian D Sykes, David Westaway

Publikováno v: PLoS Pathogens, Vol 14, Iss 1, p e1006826 (2018)

To explore pathogenesis in a young Gerstmann-Sträussler-Scheinker Disease (GSS) patient, the corresponding mutation, an eight-residue duplication in the hydrophobic region (HR), was inserted into the wild type mouse PrP gene. Transgenic (Tg) mouse l

Externí odkaz: https://doaj.org/article/a81806809d1741fdbe1f6cd4fbdb15ec

Still Heart Encodes a Structural HMT, SMYD1b, with Chaperone-Like Function during Fast Muscle Sarcomere Assembly.

Autor: Kendal Prill, Pamela Windsor Reid, Serene L Wohlgemuth, David B Pilgrim

Publikováno v: PLoS ONE, Vol 10, Iss 11, p e0142528 (2015)

The vertebrate sarcomere is a complex and highly organized contractile structure whose assembly and function requires the coordination of hundreds of proteins. Proteins require proper folding and incorporation into the sarcomere by assembly factors,

Externí odkaz: https://doaj.org/article/0ad267fc09e54f51ac43e0df9cf9583f

Resident memory CD4 ⁺ T lymphocytes mobilize from bone marrow to contribute to a systemic secondary immune reaction.

Autor: Cendón C; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Du W; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Durek P; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Liu YC; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Alexander T; Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany., Serene L; Department of Biological Sciences, University of Notre Dame, Notre Dame, Indiana, USA., Yang X; Otto-Warburg-Laboratory, Computational Epigenomics, Max Planck Institute for Molecular Genetics, Berlin, Germany., Gasparoni G; Department of Genetics, University of Saarland (UdS), Saarbrücken, Germany., Salhab A; Department of Genetics, University of Saarland (UdS), Saarbrücken, Germany., Nordström K; Department of Genetics, University of Saarland (UdS), Saarbrücken, Germany., Lai T; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Schulz AR; Mass Cytometry, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Rao A; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Heinz GA; Therapeutic Gene Regulation, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Stefanski AL; Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany., Claußnitzer A; Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany., Siewert K; Department of Chemical and Product Safety, German Federal Institute for Risk Assessment, Berlin, Germany., Dörner T; Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany., Chang HD; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany.; Schwiete-Laboratory for Microbiota and Inflammation, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Volk HD; Institute for Medical Immunology, Charité-Universitätsmedizin Berlin, Berlin, Germany.; BIH Center for Regenerative Therapies (BCRT), Charité-Universitätsmedizin Berlin, Berlin, Germany., Romagnani C; Innate Immunity, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany.; Medical Department/Gastroenterology, Infectiology and Rheumatology, Charité-Universitätsmedizin Berlin, Berlin, Germany., Qin Z; Key Laboratory of Protein and Peptide Pharmaceuticals, Institute of Biophysics, Chinese Academy of Sciences, Bejing, P. R. China.; Medical Research Center, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, P. R. China., Hardt S; Center for Musculoskeletal Surgery, Charité-Universitätsmedizin Berlin, Berlin, Germany., Perka C; Center for Musculoskeletal Surgery, Charité-Universitätsmedizin Berlin, Berlin, Germany., Reinke S; BIH Center for Regenerative Therapies (BCRT), Charité-Universitätsmedizin Berlin, Berlin, Germany., Walter J; Department of Genetics, University of Saarland (UdS), Saarbrücken, Germany., Mashreghi MF; Therapeutic Gene Regulation, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany.; BIH Center for Regenerative Therapies (BCRT), Charité-Universitätsmedizin Berlin, Berlin, Germany., Thurley K; Systems Biology of Inflammation, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany.; Institute for Theoretical Biology, Humboldt University, Berlin, Germany., Radbruch A; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany., Dong J; Cell Biology, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Institute of the Leibniz Association, Berlin, Germany.

Publikováno v: European journal of immunology [Eur J Immunol] 2022 May; Vol. 52 (5), pp. 737-752. Date of Electronic Publication: 2022 Mar 14.

A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.

Autor: Mercer, Robert C. C., Daude, Nathalie, Dorosh, Lyudmyla, Fu, Ze-Lin, Mays, Charles E., Gapeshina, Hristina, Wohlgemuth, Serene L., Acevedo-Morantes, Claudia Y., Yang, Jing, Cashman, Neil R., Coulthart, Michael B., Pearson, Dawn M., Joseph, Jeffrey T., Wille, Holger, Safar, Jiri G., Jansen, Gerard H., Stepanova, Maria, Sykes, Brian D., Westaway, David

Publikováno v: PLoS Pathogens; 1/16/2018, Vol. 14 Issue 1, p1-33, 33p

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