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American Journal of Pathology, Vol 141, 241-247, Copyright © 1992 by American Society for Investigative Pathology
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JM Campistol, M Sole, JA Bombi, R Rodriguez, E Mirapeix, J Munoz-Gomez and OW Revert
Department of Nephrology, Hospital Clinic, University of Barcelona, School of Medicine, Spain.
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.
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