Proteins of the systemic amyloidoses
- 1 January 1994
- journal article
- review article
- Published by Wolters Kluwer Health in Current Opinion in Rheumatology
- Vol. 6 (1) , 55-67
- https://doi.org/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.Keywords
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