Deposition of kappa and lambda light chains in amyloid filaments of dialysis-related amyloidosis.

Journal of the American Society of Nephrology : JASN
D BrancaccioG Coggi

Abstract

beta 2-Microglobulin (beta 2m) is considered to be the amyloidogenic precursor in dialysis-related amyloidosis, although the implication of other relevant cofactors in the pathogenesis of this disease has also been hypothesized. It is conceivable that substances found in amyloid deposits might represent something more than simple codeposition, possibly playing a pathogenic role in amyloidogenesis. Along these lines, a detailed analysis of the protein composition of amyloid fibrils purified from synovial material surgically obtained from nine patients on long-term dialysis was carried out. By the use of sodium dodecyl sulfate-polyacrylamide gel electrophoresis, several other protein components, in addition to beta 2m, were found. These were characterized by NH2 amino-terminal sequencing and immunoblotting. In fibrils obtained by water extraction, which fulfill the electron microscopy criteria of highly pure amyloid material, polyclonal kappa and lambda light chains were detected with a concentration of 15 micrograms/mL in the water extraction material; the beta 2m concentration was 200 micrograms/mL. Light microscopy immunohistochemistry was performed on samples from five patients. Amyloid deposits reacted with anti-beta 2m, and...Continue Reading

Citations

Aug 22, 2001·Protein Science : a Publication of the Protein Society·D P Smith, S E Radford
Feb 13, 2001·Kidney International. Supplement·J Floege, M Ketteler
Nov 18, 1997·European Journal of Biochemistry·M StoppiniG Ferri

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