Results 61 to 70 of about 862,879 (325)

Hyperfunctional complement C3 promotes C5-dependent atypical hemolytic uremic syndrome in mice

Journal of Clinical Investigation, 2019
Atypical hemolytic uremic syndrome (aHUS) is frequently associated in humans with loss-of-function mutations in complement-regulating proteins or gain-of-function mutations in complement-activating proteins.
K. Smith-Jackson, K. Smith-Jackson, Yi Yang, Harriet Denton, I. Pappworth, K. Cooke, P. Barlow, J. Atkinson, M. Liszewski, M. Pickering, David Kavanagh, David Kavanagh, H. Cook, K. Marchbank, K. Marchbank   +14 more
semanticscholar   +1 more source

Expanding Hereditary Spastic Paraplegias Limits: Biallelic SPAST Variants in Cerebral Palsy Mimics

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Hereditary spastic paraplegias (HSP) are rare neurodegenerative disorders marked by spasticity and lower limb weakness. The most common type, SPG4, is usually autosomal dominant and caused by SPAST gene variants, typically presenting as pure HSP.
Gregorio A. Nolasco, Mònica Roldán, Yalda Jamshidi, Ioannis Georvasilis, Rocío Jadraque Rodríguez, Reza Boostani, Ali Shoeibi, Lluís Armengol, Anna Codina, Ehsan Ghayoor Karimiani, Cristina Hernando‐Davalillo, Loreto Martorell, María Luisa Ramírez Almaraz, Jordi Muchart, Carlos Ortez, Andrés Nascimento, Roser Urreizti, Daniel Natera‐de Benito, Mercedes Serrano   +18 more
wiley   +1 more source

Impact of Stress‐Induced Hyperglycemia on In‐Hospital Medical Complications in Patients With Acute Stroke: From a Large‐Scale Nationwide Longitudinal Registry

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Aims This study aimed to explore the relationship between stress‐induced hyperglycemia (SIH) and in‐hospital medical complications in patients with acute stroke. Methods We enrolled 865,765 patients with acute stroke from the Chinese Stroke Center Alliance cohort.
Xintong Song, Yi Ju, Hongqiu Gu, Zhikai Zhu, Zixiao Li, Qian Zhang, Xingquan Zhao   +6 more
wiley   +1 more source

Low Serum Complement C3 Levels at Diagnosis of Renal ANCA-Associated Vasculitis Is Associated with Poor Prognosis

PLoS ONE, 2016
Background Recent studies have demonstrated the key role of the complement alternative pathway (cAP) in the pathophysiology of experimental ANCA-associated vasculitis (AAV). However, in human AAV the role of cAP has not been extensively explored.
J. Augusto, V. Langs, J. Demiselle, C. Lavigne, Benoît Brilland, Agnès Duveau, C. Poli, A. Chevailler, A. Croué, F. Tollis, J. Sayegh, J. Subra   +11 more
semanticscholar   +1 more source

Treating lupus nephritis patients to lupus low disease activity reduces renal relapse and preserves long‐term kidney function

Arthritis Care &Research, Accepted Article.
Objective Lupus low disease activity state (LLDAS) is a validated treatment target in systemic lupus erythematosus (SLE) but limited studies have explored the role of LLDAS in lupus nephritis (LN). This study aims to investigate the frequency and predictors of LLDAS attainment, and its benefit on LN relapse and renal function preservation in patients ...
Chak Kwan Cheung, Desmond YH Yap, KL Lee, Philip H Li, Iris YK Tang, Chak Sing Lau, Shirley CW Chan   +6 more
wiley   +1 more source

C3-Glomerulopathy Autoantibodies Mediate Distinct Effects on Complement C3- and C5-Convertases [PDF]

Frontiers in Immunology, 2019
C3 glomerulopathy (C3G) is a severe kidney disease, which is caused by defective regulation of the alternative complement pathway. Disease pathogenesis is heterogeneous and is caused by both autoimmune and genetic factors. Here we characterized IgG autoantibodies derived from 33 patients with autoimmune C3 glomerulopathy.
Peter F. Zipfel, Peter F. Zipfel, Ina Löschmann, Christine Skerka, S Lindner, Michael Kirschfink, Gesa Schalk, Bo Nilsson, Sara Afonso, Kristina Nilsson Ekdahl, Andrea Hartmann, Sandra Habbig, Lutz T. Weber, Fei Zhao   +13 more
openaire   +5 more sources

Serum soluble mediator signatures of lupus nephritis: histological features and response to treatment

Arthritis Care &Research, Accepted Article.
Objective Lupus nephritis (LN) management remains challenging, and novel noninvasive biomarkers are needed. This study quantified serum soluble mediators in the Accelerating Medicines Partnership (AMP) LN cohort to identify biomarkers of histological features and treatment response.
Andrea Fava, Catriona A. Wagner, Carla J. Guthridge, Susan Macwana, Wade DeJager, Melissa E. Munroe, Peter Izmirly, H. Michael Belmont, Betty Diamond, Anne Davidson, Paul J. Utz, Michael H Weisman, Philip M Carlucci, Maria Dall'Era, Kenneth Kalunian, Chaim Putterman, Jennifer Anolik, Jennifer L Barnas, David Wofsy, Diane Kamen, Richard A Furie, Deepak A Rao, the Accelerating Medicines Partnership (AMP) RA/SLE Network, Michelle Petri, Joel M. Guthridge, Jill Buyon, Judith A. James   +26 more
wiley   +1 more source

Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come

Clinical and Developmental Immunology, 2012
Inherited and acquired dysregulation of the complement alternative pathway plays an important role in multiple renal diseases. In recent years, the identification of disease-causing mutations and genetic variants in complement regulatory proteins has ...
Saskia F. Heeringa, Clemens D. Cohen
doaj   +1 more source

Complement C3 exacerbates renal interstitial fibrosis by facilitating the M1 macrophage phenotype in a mouse model of unilateral ureteral obstruction.

AJP - Renal Physiology, 2019
The impact of the renal microenvironment on macrophage phenotypes determination can contribute to the progression or resolution of renal fibrosis. While the complement proteins affect macrophage polarization, whether complement component 3 (C3) can ...
Jiong Cui, Xiaoting Wu, Yankun Song, Yi Chen, Jianxin Wan   +4 more
semanticscholar   +1 more source

A Spatially Resolved View on the Aging Substantia nigra: An Exploratory Proteomic Study

Advanced Biology, EarlyView.
Although aging is the most important risk factor for several neurodegenerative diseases, the molecular effects of physiological aging are still understudied. By applying spatially‐resolved proteomic analyses of the human substantia nigra pars compacta, alterations in vesicular trafficking and mitochondrial proteins are observed, as well as reduced ...
Britta Eggers, Maximilian Hausherr, Michel Lim, Karin Schork, Bilhan Karacora, Robin Grugel, Martin Eisenacher, Isabel Gil Aldea, Peter Riederer, Manfred Gerlach, Katrin Marcus   +10 more
wiley   +1 more source