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We identified a novel, homozygous, loss-of-function mutation (p.Pro445Leufs*11) in the C1R gene. Using the Sanger method of DNA sequencing in 14 family members, we confirmed the presence of the mutation in 4 patients with early-onset systemic lupus erythematosus and in an asymptomatic 9-year-old girl. Complement levels were low in sera from patients with truncated C1r protein.
Periodontal Ehlers-Danlos Syndrome in at least the great majority of cases results from specific classes of heterozygous mutations in C1R and C1S (显示 C1S 蛋白).
We confirmed increased levels of C1R and VTN (显示 VTN 蛋白) in sera from patients with Joint hypermobility syndrome by western blot analyses
C1q exists as the C1 complex (C1qC1r2C1s2), and C1q binding to ligands activates the C1r/C1s (显示 C1S 蛋白) proteases. Incubation of nucleoli with C1 caused degradation of the nucleolar proteins nucleolin (显示 NCL 蛋白) and nucleophosmin 1 (显示 NPM1 蛋白). T
C1r specificity is well suited to its cleavage targets and that efficient cleavage of C1s (显示 C1S 蛋白) is achieved through both active site and exosite contributions.
Analysis of its interaction properties by surface plasmon resonance shows that rC1q retains the ability of serum C1q to associate with the C1s (显示 C1S 蛋白)-C1r-C1r-C1s (显示 C1S 蛋白) tetramer, to recognize physiological C1q ligands such as IgG and pentraxin 3 (显示 PTX3 蛋白)
a structural rearrangement as a switch between functional states of human C1r
These results provide further structural insights into the architecture of the C1 complex, and the interactions between C1r and C1s (显示 C1S 蛋白).
The modular C1r protein is the first protease activated in the classical complement pathway, a key component of innate immunity.
Detailed mapping of C1q post-translational modifications and insights into the C1r/C1s (显示 C1S 蛋白) binding sites.
C1r B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system.
complement C1r subcomponent
, complement C1r
, complement component 1, r subcomponent
, complement component 1 subcomponent r