Storage of the complement components C4, C3, and C 3-activator in the human liver as PAS-negative globular hyaline bodies.
Liver biopsies of a 58-year-old clinically healthy patient with a hepatomegaly and intracisternal PAS-negative globular hyaline bodies were immunofluorescent-optically examined for the content of the complement components C 1 q, C 4, C 9, C 1-inactivator, C 3-activator. Further examinations were performed for fibrinogen, IgG, IgA, IgM, IgD, IgE, L-chain (type chi and lambda), alpha 1-antitrypsin, alpha 1-fetoprotein, alpha 1- and alpha 2-glycoprotein, cholinesterase, ceruloplasmin, myoglobin, hemopexin, HBsAg and HBsAg. Th inclusion bodies reacted with antisera against the complement components C 4, C 3 and C 3-activator, as also identified by double immunofluorescence. Probably this is a disturbance of the protein metabolism of the liver cell with abnormal complement storage in the presence of normal total complement and normal complement components in the serum.
D003166:Complement Activating Enzymes / Q000378:metabolism*; D003176:Complement C3 / Q000378:metabolism*; D050577:Complement C3-C5 Convertases / Q000378:metabolism*; D003181:Complement C4 / Q000378:metabolism*; D006801:Humans; D002479:Inclusion Bodies / Q000276:immunology; D008099:Liver / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010503:Periodic Acid-Schiff Reaction
D003176:Complement C3; D003181:Complement C4; D003166:Complement Activating Enzymes; D050577:Complement C3-C5 Convertases