Proteins of the systemic amyloidoses.
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.
Cohen
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Arthritis and Amyloid Center, Boston University School of Medicine, MA 02118.
D000328:Adult; D000595:Amino Acid Sequence; D000682:Amyloid / Q000235:genetics*; D000686:Amyloidosis / Q000209:etiology / Q000235:genetics*; D000818:Animals; D006801:Humans; D008969:Molecular Sequence Data; D009154:Mutation; D011228:Prealbumin / Q000235:genetics; D000685:Serum Amyloid A Protein / Q000235:genetics; D001613:beta 2-Microglobulin / Q000235:genetics
D000682:Amyloid; D011228:Prealbumin; D000685:Serum Amyloid A Protein; D001613:beta 2-Microglobulin