MOLECULAR BASIS OF β2-MICROGLOBULIN AMYLOIDOSIS



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Abstract

Beta2-microglobulin am yloidosis (Aβ2M -amyloidosis) - is complication of chronic hemodialysis, resulting from accumulation of amyloid deposits in the patient’s tissues. The major component of the amyloid is presented by beta2-microglobulin (β2M) fibrils. Normally β2M is excreted by kidneys, but in patients with end-stage renal disease β2M concentration is continuously increased due to impaired renal excretion. High β2M serum concentration is necessary but not sufficient for the development of Aβ2M-amyloidosis. Other factors resulting in the β2M polymerization and the deposition of amyloid fibrils are not clear, and in present time they the subject of in vivo and in vitro studies. Despite the clinical importance and fairly high incidence of this disease, in Russian literature these issue is gaining very little attention. In particular, we do not know works, descriding in detail the molecular aspect of the Aß2M amyloidosis. In this review, we have attempted to summarize the literature data and the results obtained in our laboratory regarding to the biochemical processes underlying the abnormal fibrilogenesis of β2M. Individually it is written about the problems of in vitro simulation, diagnosis, and immunological aspects of the disease. The clinical manifestations of Aβ2M amyloidosis are briefly summarized.

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About the authors

D S Polyakov

Institute of Experimental Medicine of the North-West Branch of the Russian Academy of Medical Sciences

Email: ravendoctor@mail.ru
St.-Petersburg, Russia

M M Shavlovsky

Institute of Experimental Medicine of the North-West Branch of the Russian Academy of Medical Sciences

St.-Petersburg, Russia

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