In situ complement activation in porcine membranoproliferative glomerulonephritis type II

摘要: In situ complement activation in porcine membranoproliferative glomerulonephritis type II. Pigs genetically deficient factor H all develop lethal (MPGN) II characterized by massive glomerular deposits of complement, intramembranous dense deposits, and mesangial hypercellularity. To elucidate the chronological relationship between these changes, to precisely determine localization we studied kidney specimens from H-deficient piglets at different ages fetal life until terminal failure had developed. Deposits C3 complex localized within basement membrane (GBM) were present already fetuses, without concurrent or Incipient subendothelial containing appeared no earlier than four days after birth, older with established MPGN also contained large amounts as detected immune electron microscopy. Onset coincided pronounced hypercellularity expansion, compromising capillary patency. Formation wall double contours microscopic evidence laminar disintegration deposits. Complement was deposited matrix, but not on cells. We conclude that components alternative pathways have access into GBM matrix. absence H, is spontaneously activated locations resulting deposit formation. It proposed dysfunction may play an essential role even human