Results 21 to 30 of about 5,097 (156)
Clinical Case Reports, 2022 Periodontal Ehlers‐Danlos syndrome (pEDS) is a rare disorder caused by heterozygous mutations in complement 1 subunit genes C1R and C1S. To date, 148 cases have been described in the literature.We describe a case of a suspected de novo‐mutation of pEDS ...
Manfred Nilius +5 more
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No prominent role for complement C1-esterase inhibitor in Marfan syndrome mice
Vascular Biology, 2022 Marfan syndrome (MFS) is a connective tissue disorder causing aortic aneurysm formation. Currently, only prophylactic aortic surgery and blood pressure-lowering drugs are available to reduce the risk of aortic rupture.
Stijntje Hibender +13 more
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Complement C1s as a diagnostic marker and therapeutic target: Progress and propective
Frontiers in Immunology, 2022 The molecules of the complement system connect the effectors of innate and adaptive immunity and play critical roles in maintaining homeostasis. Among them, the C1 complex, composed of C1q, C1r, and C1s (C1qr2s2), is the initiator of the classical ...
Jun Ye +4 more
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A Structural Basis for Inhibition of the Complement Initiator Protease C1r by Lyme Disease Spirochetes [PDF]
The Journal of Immunology, 2021 Abstract Complement evasion is a hallmark of extracellular microbial pathogens such as Borrelia burgdorferi, the causative agent of Lyme disease. Lyme disease spirochetes express nearly a dozen outer surface lipoproteins that bind complement components and interfere with their native activities.
Ryan J Garrigues +4 more
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The axis of complement C1 and nucleolus in antinuclear autoimmunity
Frontiers in Immunology, 2023 Antinuclear autoantibodies (ANA) are heterogeneous self-reactive antibodies that target the chromatin network, the speckled, the nucleoli, and other nuclear regions.
Shan Wu +15 more
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Characteristics of complement subcomponents C1r and C1s synthesized by Hep G2 cells [PDF]
Biochemical Journal, 1986 The association and activation states of complement subcomponents C1r and C1s biosynthesized by Hep G2 cells were studied. C1r and C1s are secreted in stoichiometric amounts; in the presence of Ca2+ they are associated in a complex that sediments similarly to plasma C1r2-C1s2.
A, Reboul, J C, Bensa, M G, Colomb
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Structure of the C1r–C1s interaction of the C1 complex of complement activation [PDF]
Proceedings of the National Academy of Sciences, 2018 SignificanceC1 is a large complex that triggers the destruction of invading pathogens via lysis or by stimulation of innate and adaptive immune processes. It is composed of C1q, a protein with a bouquet-like architecture, together with a tetramer assembled from two copies each of the serine proteases C1r and C1s, which activate when C1q binds to a ...
Jamal O. M. Almitairi +9 more
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Biomolecules, 2021 We report an extremely rare case of combined classical and periodontal Ehlers−Danlos syndrome (EDS) with early severe periodontitis and a generalized lack of attached gingiva.
Friedrich Stock +6 more
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Structures of the MASP Proteases and Comparison with Complement C1r and C1s [PDF]
, 2021 Several recognition proteins of the defence collagen family associate with proteases to initiate the complement cascade. The associated proteases, which are the subject of this review, mediate the proteolytic activation trigger. MBL-associated serine proteases (MASPs) mainly activate the lectin complement pathway (LP), while C1r and C1s activate the ...
Gaboriaud, Christine +2 more
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PLoS Pathogens, 2019 The carboxy-terminal domain of the BBK32 protein from Borrelia burgdorferi sensu stricto, termed BBK32-C, binds and inhibits the initiating serine protease of the human classical complement pathway, C1r.
Jialei Xie +5 more
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