Prealbumin variants in the amyloid fibrils of Swedish familial amyloidotic polyneuropathy

Clin Exp Immunol. 1987 Sep;69(3):695-701.

Abstract

Amino-acid sequence analysis of an amyloid fibril protein from a patient with Swedish familial amyloidotic polyneuropathy showed homology with prealbumin but with heterogeneous N-terminal deletions. One-third of the molecules had the same amino acid substitution, methionine for valine in position 30, as in familial amyloidosis of Portuguese, Japanese and Swedish-American type. A protein with the same antigenic properties and size was found in the fibrils of two other patients with Swedish FAP while the amyloid fibrils in two further patients predominantly contained a smaller prealbumin-derived protein. Cyanogen bromide cleavage of this protein revealed no evidence for a methionine residue in position 30.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amino Acid Sequence
  • Amino Acids / analysis
  • Amyloid / analysis*
  • Amyloidosis / genetics*
  • Amyloidosis / metabolism
  • Electrophoresis, Polyacrylamide Gel
  • Female
  • Humans
  • Immunodiffusion
  • Male
  • Middle Aged
  • Nervous System Diseases / genetics*
  • Nervous System Diseases / metabolism
  • Prealbumin / analysis*

Substances

  • Amino Acids
  • Amyloid
  • Prealbumin
  • amyloid prealbumin