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Complement C1r is a serine protease and a key component of the classical pathway of the complement system. It combines with C1q and C1s to form the C1 complex, which initiates the classical complement cascade. Upon binding to immune complexes, C1r autoactivates and cleaves C1s, triggering downstream cleavage events. Mutations in C1R have been associated with Ehlers-Danlos syndrome periodontal type.
Cleaves and activates proenzyme C1s
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