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COMPLEMENT COMPONENT C1r/C1s DEFICIENCY

Known as: C1r/C1s DEFICIENCY 
Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will… 
National Institutes of Health

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Complement deficiency

Papers overview

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2000
2000
The Cleavage of Two C1s Subunits by a Single Active C1r Reveals Substantial Flexibility of the C1s-C1r-C1r-C1s Tetramer in the C1 Complex1
The activation of the C1s-C1r-C1r-C1s tetramer in the C1 complex, which involves the cleavage of an Arg-Ile bond in the catalytic… 
1994
1994
Activation of human complement serine-proteinase C1r is down-regulated by a Ca(2+)-dependent intramolecular control that is released in the C1 complex through a signal transmitted by C1q.
The activation of human C1, a Ca(2+)-dependent complex proteinase comprising a non-enzymic protein, C1q, and two serine… 
1990
1990
Trimer and tetramer complexes containing C1 esterase inhibitor, C1r and C1s, in serum and synovial fluid of patients with rheumatic disease.
1986
1986
C1 activation, with C1q in excess of functional C1 in synovial fluid from patients with rheumatoid arthritis.
Free Clq, in functionally active form was present in increased amounts in the synovial fluid of patients with rheumatoid… 
Highly Cited
1985
Highly Cited
1985
Sequential studies of complement activation in systemic lupus erythematosus.
C1 and C3 activation, measured as C1r-C1s-C1 inactivator C1s-C1r-C1IA complexes in serum and circulating C3d were studied in… 
1985
1985
Lyme Disease in a 12‐year‐old Girl
ABSTRACT. We report the case of a 12‐year‐old girl with erythema chronicum migrans, aseptic meningitis and knee arthralgia. Rise… 
1985
1985
Correlation among complement activation, protease inhibitors, and clinical course in acute pancreatitis in man.
Changes in complement levels and protease inhibitors were measured in plasma/serum and peritoneal fluid during 15 attacks of… 
1982
1982
Fluid phase activation of proenzymic C1r purified by affinity chromatography.
1981
1981
Complement in pneumococcal infections with varying degrees of severity.
Complement component levels (Clq, Cls, C4, C3, factor B and properdin) and C1 subcomponent complexes (C1r-C1s, C1-r-C1-, C1-r-C1… 
1980
1980
Complement activation, circulating C1q binding substances and inflammatory activity in rheumatoid arthritis: relations and changes on suppression of inflammation.
Patients with rheumatoid arthritis were treated with podophyllotoxin derivatives (PTD) or with cyclophosphamide. Increased… 
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