In vitro spontaneous synthesis of beta 2-microglobulin amyloid fibrils in peripheral blood mononuclear cell culture.
beta 2-microglobulin-related amyloidosis (A beta 2M), in long-term dialysis patients, is a new and frequent complication for which the pathogenesis remains unknown. The authors documented, by light and high resolution electron microscopy, the spontaneous polymerization of beta 2-microglobulin to amyloid fibrils in mononuclear cell culture supernatants from dialysis patients. These data provide significant information about the pathogenesis of dialysis-amyloidosis, revealing an unusual and different fibrillogenic mechanism for beta 2-microglobulin and dialysis-amyloidosis than for other forms of amyloidosis. beta 2-microglobulin does not appear to require a proteolytic process before polymerization into amyloid fibrils and deposits. This study represents the first cell culture system in which beta 2-microglobulin amyloid fibrils have been spontaneously created.
Campistol
J M
JM
Department of Nephrology, Hospital Clinic, University of Barcelona, School of Medicine, Spain.
Solé
M
M
Bombi
J A
JA
Rodriguez
R
R
Mirapeix
E
E
Muñoz-Gomez
J
J
Revert
O W
OW
D000682:Amyloid / Q000032:analysis / Q000378:metabolism*; D002478:Cells, Cultured; D006801:Humans; D007963:Leukocytes, Mononuclear / Q000737:chemistry / Q000378:metabolism* / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D011108:Polymers; D001613:beta 2-Microglobulin / Q000032:analysis / Q000378:metabolism*
D000682:Amyloid; D011108:Polymers; D001613:beta 2-Microglobulin