Zobrazeno 1 - 10
of 65
pro vyhledávání: '"Richard J H, Smith"'
Autor:
Cobey J. H. Donelson, Nicolo Ghiringhelli Borsa, Amanda O. Taylor, Richard J. H. Smith, Yuzhou Zhang
Publikováno v:
Frontiers in Immunology, Vol 15 (2024)
The regulatory serine protease, complement factor I (FI), in conjunction with one of its cofactors (FH, C4BP, MCP, or CR1), plays an essential role in controlling complement activity through inactivation of C3b and C4b. The functional impact by misse
Externí odkaz:
https://doaj.org/article/221d1b499b1f4d6697c25441e7dda17e
Autor:
Sofiya Pisarenka, Nicole C. Meyer, Xue Xiao, Renee Goodfellow, Carla M. Nester, Yuzhou Zhang, Richard J. H. Smith
Publikováno v:
Frontiers in Immunology, Vol 13 (2023)
IntroductionC3 glomerulopathies (C3G) are ultra-rare complement-mediated diseases that lead to end-stage renal disease (ESRD) within 10 years of diagnosis in ~50% of patients. Overactivation of the alternative pathway (AP) of complement in the fluid
Externí odkaz:
https://doaj.org/article/278dc68f339840d88429924d9bfc4547
Autor:
Yuzhou Zhang, Renee X. Goodfellow, Nicolo Ghiringhelli Borsa, Hannah C. Dunlop, Stephen A. Presti, Nicole C. Meyer, Dingwu Shao, Sarah M. Roberts, Michael B. Jones, Gabriella R. Pitcher, Amanda O. Taylor, Carla M. Nester, Richard J. H. Smith
Publikováno v:
Frontiers in Immunology, Vol 13 (2022)
C3 glomerulopathy (C3G) and atypical hemolytic uremic syndrome (aHUS) are two rare diseases caused by dysregulated activity of the alternative pathway of complement secondary to the presence of genetic and/or acquired factors. Complement factor I (FI
Externí odkaz:
https://doaj.org/article/29409b69ea43436987bb7a1d0d8209d0
Autor:
Markus A. Loeven, Marissa L. Maciej-Hulme, Cansu Yanginlar, Melanie C. Hubers, Edwin Kellenbach, Mark de Graaf, Toin H. van Kuppevelt, Jack Wetzels, Ton J. Rabelink, Richard J. H. Smith, Johan van der Vlag
Publikováno v:
Frontiers in Immunology, Vol 12 (2021)
Complement dysregulation is characteristic of the renal diseases atypical hemolytic uremic syndrome (aHUS) and complement component 3 glomerulopathy (C3G). Complement regulatory protein Factor H (FH) inhibits complement activity, whereas FH-related p
Externí odkaz:
https://doaj.org/article/cb5d110dffb7463ebb487d15131e30fc
Autor:
Rossella Piras, Matteo Breno, Elisabetta Valoti, Marta Alberti, Paraskevas Iatropoulos, Caterina Mele, Elena Bresin, Roberta Donadelli, Paola Cuccarolo, Richard J. H. Smith, Ariela Benigni, Giuseppe Remuzzi, Marina Noris
Publikováno v:
Frontiers in Genetics, Vol 12 (2021)
C3 Glomerulopathy (C3G) and Immune Complex-Mediated Membranoproliferative glomerulonephritis (IC-MPGN) are rare diseases characterized by glomerular deposition of C3 caused by dysregulation of the alternative pathway (AP) of complement. In approximat
Externí odkaz:
https://doaj.org/article/bf5651269e7640a2b06033b39950716b
Autor:
Yuzhou Zhang, Nicolo Ghiringhelli Borsa, Dingwu Shao, Arthur Dopler, Michael B. Jones, Nicole C. Meyer, Gabriella R. Pitcher, Amanda O. Taylor, Carla M. Nester, Christoph Q. Schmidt, Richard J. H. Smith
Publikováno v:
Frontiers in Immunology, Vol 11 (2020)
Factor H (FH), a member of the regulators-of-complement-activation (RCA) family of proteins, circulates in human plasma at concentrations of 180–420 mg/L where it controls the alternative pathway (AP) of complement in the fluid phase and on cell su
Externí odkaz:
https://doaj.org/article/0283cd6e288a4ae782754f13d2aacef7
Autor:
Andrew S, Bomback, David, Kavanagh, Marina, Vivarelli, Matthias, Meier, Yaqin, Wang, Nicholas J A, Webb, Angelo J, Trapani, Richard J H, Smith
Publikováno v:
Kidney International Reports. 7:2150-2159
Complement 3 glomerulopathy (C3G) is a rare kidney disease characterized by dysregulation of the alternative pathway (AP) of the complement system. About 50% of patients with C3G progress to kidney failure within 10 years of diagnosis. Currently, the
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9459cb8f6043be21a7930696affaa654
https://doi.org/10.1016/j.ekir.2022.07.004
https://doi.org/10.1016/j.ekir.2022.07.004
Publikováno v:
American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 190:344-357
C3 glomerulopathy (C3G) describes a pathologic pattern of injury diagnosed by renal biopsy. It is characterized by the dominant deposition of the third component of complement (C3) in the renal glomerulus as resolved by immunofluorescence microscopy.
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3bb6d5113bb0d26e7c9074c6371712c8
https://doi.org/10.1002/ajmg.c.31986
https://doi.org/10.1002/ajmg.c.31986
Publikováno v:
Frontiers in Immunology, Vol 10 (2019)
C3 Glomerulopathy (C3G) is a renal disease mediated primarily by dysregulation of the alternative pathway of complement. Complement is the cornerstone of innate immunity. It targets infectious microbes for destruction, clears immune complexes, and ap
Externí odkaz:
https://doaj.org/article/c2ee36351c9c4e24872c8441dbc779ab
Autor:
Farrel J Buchinsky, William L Valentino, Nicole Ruszkay, Evan Powell, Craig S Derkay, Riaz Y Seedat, Virgilijus Uloza, Frederik G Dikkers, David E Tunkel, Sukgi S Choi, Anthony J Mortelliti, Paolo Campisi, Juan C Ospina, Adam J Donne, Robert T Sataloff, Stephen F Conley, John E McClay, Ellen M Friedman, Lisa Elden, Dale A Tylor, Clark A Rosen, Libby J Smith, Graeme J Copley, David E Karas, John M Schweinfurth, Charles M Myer, Brian J Wiatrak, Joseph E Dohar, Steven E Sobol, Robert W Bastian, Richard J H Smith, Marshall E Smith, Abebe M Wassie, James C Post, Garth D Ehrlich
Publikováno v:
PLoS ONE, Vol 14, Iss 6, p e0216697 (2019)
BackgroundRecurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the
Externí odkaz:
https://doaj.org/article/9ae6df7ce2f54e3f81c43f2b54a50fdf