Fibrillary glomerulonephritis related to serum fibrillar immunoglobulin-fibronectin complexes.
Fibrillary glomerulonephritis is a disease of uncertain origin and pathogenesis characterized by nonamyloidotic fibrils in glomeruli. We report immunohistological, immunochemical, and biochemical studies of a serum fibrillar cryoprecipitate obtained from a patient with fibrillary glomerulonephritis, that formed on prolonged storage at 4 degrees C. By Western blot and amino acid sequence analysis, the cryoprecipitated fibril components consisted of immunoglobulins, heavy chains gamma and mu, light chains kappa and lambda, and fibronectin, similar to the proteins identified by immunofluorescence and immunoelectron microscopy in the glomerular fibrils. These findings support the hypothesis that serum precursors may be the source of the fibrillar deposits and suggest a role for immunoglobulin-fibronectin complexes in the pathogenesis of fibrillary glomerulonephritis.
Rostagno
A
A
Department of Pathology, New York University Medical Center, New York 10016, USA.
Vidal
R
R
Kumar
A
A
Chuba
J
J
Niederman
G
G
Gold
L
L
Frangione
B
B
Ghiso
J
J
Gallo
G
G
D000936:Antigen-Antibody Complex / Q000097:blood* / Q000378:metabolism; D015153:Blotting, Western; D003450:Cryoglobulins / Q000032:analysis* / Q000378:metabolism; D005260:Female; D005353:Fibronectins / Q000097:blood* / Q000378:metabolism; D005921:Glomerulonephritis / Q000097:blood / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007136:Immunoglobulins / Q000097:blood* / Q000378:metabolism; D007678:Kidney Glomerulus / Q000737:chemistry / Q000473:pathology*; D008854:Microscopy, Electron; D016253:Microscopy, Immunoelectron; D008875:Middle Aged
D000936:Antigen-Antibody Complex; D003450:Cryoglobulins; D005353:Fibronectins; D007136:Immunoglobulins