Proteins of the systemic amyloidoses

Curr Opin Rheumatol. 1994 Jan;6(1):55-67. doi: 10.1097/00002281-199401000-00010.

Abstract

Significant advances were made this year in the understanding of serum amyloid A isotypes and in the definition of different amyloid light-chain proteins. Increasing numbers of hereditary amyloid-related transthyretin mutations have been reported (more than 30 to date). Two new hereditary amyloid proteins in several different kinships have appeared, ie, fibrinogen A alpha and lysozyme, each with a single point mutation. Both were found in patients with non-neurogenic hereditary amyloidosis with severe nephropathy. In islet amyloid polypeptide, the amyloid of adult-onset diabetes, the amino-acid sequence Ala-Ile-Leu-Ser at positions 25 to 28 appears to be critical for fibrillogenesis.

Publication types

  • Review

MeSH terms

  • Adult
  • Amino Acid Sequence
  • Amyloid / genetics*
  • Amyloidosis / etiology
  • Amyloidosis / genetics*
  • Animals
  • Humans
  • Molecular Sequence Data
  • Mutation
  • Prealbumin / genetics
  • Serum Amyloid A Protein / genetics
  • beta 2-Microglobulin / genetics

Substances

  • Amyloid
  • Prealbumin
  • Serum Amyloid A Protein
  • beta 2-Microglobulin