A healthy female with C3 hypocomplementemia and C3 Nephritic Factor
نویسندگان
چکیده
منابع مشابه
Mechanism of action of the C4 nephritic factor. Deregulation of the classical pathway of C3 convertase
Three mechanisms that regulate the formation and function of the classical pathway C3 convertase (C4b2a) have been elucidated: (a) an intrinsic decay of the enzyme that is temperature dependent; (b) an extrinsic decay mediated by the effect of the serum protein C4b binding protein (C4-bp); and (c) inactivation of C4b by the proteolytic action of C4b/C3b inactivator (C4b/C3bINA), which cleaves t...
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Two complex enzymes were assembled that both converted C3 to C3b, one consisting of activated properdin (P), native C3, proactivator (PA) and proactivator convertase (PAase), and the other of nephritic factor (NF) and the same three cofactors. By maintaining a critical concentration of PAase, the P-C3 convertase and the NF-C3 convertase were shown to function efficiently without formation of th...
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C3 nephritic factors are autoantibodies that prolong the half-life or prevent regulation of the alternative pathway C3 convertase, resulting in uncontrolled complement activation. They are strongly associated with renal disease but their role in pathogenesis remains controversial. Here we optimized and compared a panel of assays to identify and interrogate nephritic factor activities. Of 101 pa...
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A 5-day-old male infant with an increased dried blood spot propionylcarnitine (C3-carnitine) value of 7.93 mol/L (cutoff 6.79 mol/L) was identified by the New Jersey state newborn screening program. C3carnitine is used as a screening tool for methylmalonic and propionic acidemias, potentially fatal but treatable inborn errors of metabolism. The initial screen values provided a calculated C3:C2 ...
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ژورنال
عنوان ژورنال: Clinical Immunology
سال: 2016
ISSN: 1521-6616
DOI: 10.1016/j.clim.2016.06.001