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Proceedings of the National Academy of Sciences of the United States of America logoLink to Proceedings of the National Academy of Sciences of the United States of America
. 1990 Apr;87(7):2843–2845. doi: 10.1073/pnas.87.7.2843

Fibril in senile systemic amyloidosis is derived from normal transthyretin.

P Westermark 1, K Sletten 1, B Johansson 1, G G Cornwell 3rd 1
PMCID: PMC53787  PMID: 2320592

Abstract

The amyloid fibril in senile systemic amyloidosis (SSA), like that of familial amyloidotic polyneuropathy, is derived from transthyretin (TTR). SSA, however, is a common disease, affecting to some degree 25% of the population greater than 80 years old. In familial amyloidotic polyneuropathy, the amyloidogenesis has been considered to depend on point mutations leading to TTR variants. We show that the TTR molecule in SSA, on the other hand, has a normal primary structure. Factors other than the primary structure of TTR must therefore be important in the pathogenesis of TTR-derived amyloid.

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Selected References

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  1. Blake C. C., Geisow M. J., Oatley S. J., Rérat B., Rérat C. Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 A. J Mol Biol. 1978 May 25;121(3):339–356. doi: 10.1016/0022-2836(78)90368-6. [DOI] [PubMed] [Google Scholar]
  2. Blobel G., Dobberstein B. Transfer of proteins across membranes. I. Presence of proteolytically processed and unprocessed nascent immunoglobulin light chains on membrane-bound ribosomes of murine myeloma. J Cell Biol. 1975 Dec;67(3):835–851. doi: 10.1083/jcb.67.3.835. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Cornwell G. G., 3rd, Murdoch W. L., Kyle R. A., Westermark P., Pitkänen P. Frequency and distribution of senile cardiovascular amyloid. A clinicopathologic correlation. Am J Med. 1983 Oct;75(4):618–623. doi: 10.1016/0002-9343(83)90443-6. [DOI] [PubMed] [Google Scholar]
  4. Cornwell G. G., 3rd, Sletten K., Johansson B., Westermark P. Evidence that the amyloid fibril protein in senile systemic amyloidosis is derived from normal prealbumin. Biochem Biophys Res Commun. 1988 Jul 29;154(2):648–653. doi: 10.1016/0006-291x(88)90188-x. [DOI] [PubMed] [Google Scholar]
  5. Cornwell G. G., 3rd, Westermark P., Natvig J. B., Murdoch W. Senile cardiac amyloid: evidence that fibrils contain a protein immunologically related to prealbumin. Immunology. 1981 Nov;44(3):447–452. [PMC free article] [PubMed] [Google Scholar]
  6. Costa P. P., Figueira A. S., Bravo F. R. Amyloid fibril protein related to prealbumin in familial amyloidotic polyneuropathy. Proc Natl Acad Sci U S A. 1978 Sep;75(9):4499–4503. doi: 10.1073/pnas.75.9.4499. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Drapeau G. R. Substrate specificity of a proteolytic enzyme isolated from a mutant of Pseudomonas fragi. J Biol Chem. 1980 Feb 10;255(3):839–840. [PubMed] [Google Scholar]
  8. Dwulet F. E., Benson M. D. Characterization of a transthyretin (prealbumin) variant associated with familial amyloidotic polyneuropathy type II (Indiana/Swiss). J Clin Invest. 1986 Oct;78(4):880–886. doi: 10.1172/JCI112675. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Dwulet F. E., Benson M. D. Primary structure of an amyloid prealbumin and its plasma precursor in a heredofamilial polyneuropathy of Swedish origin. Proc Natl Acad Sci U S A. 1984 Feb;81(3):694–698. doi: 10.1073/pnas.81.3.694. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Felding P., Fex G., Westermark P., Olofsson B. O., Pitkänen P., Benson L. Prealbumin in Swedish patients with senile systemic amyloidosis and familial amyloidotic polyneuropathy. Scand J Immunol. 1985 Feb;21(2):133–140. doi: 10.1111/j.1365-3083.1985.tb01412.x. [DOI] [PubMed] [Google Scholar]
  11. Glenner G. G. Amyloid deposits and amyloidosis. The beta-fibrilloses (first of two parts). N Engl J Med. 1980 Jun 5;302(23):1283–1292. doi: 10.1056/NEJM198006053022305. [DOI] [PubMed] [Google Scholar]
  12. Gorevic P. D., Prelli F. C., Wright J., Pras M., Frangione B. Systemic senile amyloidosis. Identification of a new prealbumin (transthyretin) variant in cardiac tissue: immunologic and biochemical similarity to one form of familial amyloidotic polyneuropathy. J Clin Invest. 1989 Mar;83(3):836–843. doi: 10.1172/JCI113966. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Kanda Y., Goodman D. S., Canfield R. E., Morgan F. J. The amino acid sequence of human plasma prealbumin. J Biol Chem. 1974 Nov 10;249(21):6796–6805. [PubMed] [Google Scholar]
  14. Mita S., Maeda S., Shimada K., Araki S. Cloning and sequence analysis of cDNA for human prealbumin. Biochem Biophys Res Commun. 1984 Oct 30;124(2):558–564. doi: 10.1016/0006-291x(84)91590-0. [DOI] [PubMed] [Google Scholar]
  15. Nakazato M., Kangawa K., Minamino N., Tawara S., Matsuo H., Araki S. Revised analysis of amino acid replacement in a prealbumin variant (SKO-III) associated with familial amyloidotic polyneuropathy of Jewish origin. Biochem Biophys Res Commun. 1984 Sep 28;123(3):921–928. doi: 10.1016/s0006-291x(84)80222-3. [DOI] [PubMed] [Google Scholar]
  16. Nordlie M., Sletten K., Husby G., Ranløv P. J. A new prealbumin variant in familial amyloid cardiomyopathy of Danish origin. Scand J Immunol. 1988 Jan;27(1):119–122. doi: 10.1111/j.1365-3083.1988.tb02329.x. [DOI] [PubMed] [Google Scholar]
  17. Pitkänen P., Westermark P., Cornwell G. G., 3rd Senile systemic amyloidosis. Am J Pathol. 1984 Dec;117(3):391–399. [PMC free article] [PubMed] [Google Scholar]
  18. Pomerance A. The pathology of senile cardiac amyloidosis. J Pathol Bacteriol. 1966 Apr;91(2):357–367. doi: 10.1002/path.1700910209. [DOI] [PubMed] [Google Scholar]
  19. Pras M., Prelli F., Franklin E. C., Frangione B. Primary structure of an amyloid prealbumin variant in familial polyneuropathy of Jewish origin. Proc Natl Acad Sci U S A. 1983 Jan;80(2):539–542. doi: 10.1073/pnas.80.2.539. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Pras M., Schubert M., Zucker-Franklin D., Rimon A., Franklin E. C. The characterization of soluble amyloid prepared in water. J Clin Invest. 1968 Apr;47(4):924–933. doi: 10.1172/JCI105784. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Saraiva M. J., Birken S., Costa P. P., Goodman D. S. Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin). J Clin Invest. 1984 Jul;74(1):104–119. doi: 10.1172/JCI111390. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Sletten K., Husby G. The complete amino-acid sequence of non-immunoglobulin amyloid fibril protein AS in rheumatoid arthritis. Eur J Biochem. 1974 Jan 3;41(1):117–125. doi: 10.1111/j.1432-1033.1974.tb03251.x. [DOI] [PubMed] [Google Scholar]
  23. Sletten K., Husebekk A., Husby G. The amino acid sequence of an amyloid fibril protein AA isolated from the horse. Scand J Immunol. 1987 Jul;26(1):79–84. doi: 10.1111/j.1365-3083.1987.tb02237.x. [DOI] [PubMed] [Google Scholar]
  24. Sletten K., Westermark P., Natvig J. B. Senile cardiac amyloid is related to prealbumin. Scand J Immunol. 1980;12(6):503–506. doi: 10.1111/j.1365-3083.1980.tb00098.x. [DOI] [PubMed] [Google Scholar]
  25. Tawara S., Nakazato M., Kangawa K., Matsuo H., Araki S. Identification of amyloid prealbumin variant in familial amyloidotic polyneuropathy (Japanese type). Biochem Biophys Res Commun. 1983 Nov 15;116(3):880–888. doi: 10.1016/s0006-291x(83)80224-1. [DOI] [PubMed] [Google Scholar]
  26. Wallace M. R., Dwulet F. E., Conneally P. M., Benson M. D. Biochemical and molecular genetic characterization of a new variant prealbumin associated with hereditary amyloidosis. J Clin Invest. 1986 Jul;78(1):6–12. doi: 10.1172/JCI112573. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Wallace M. R., Dwulet F. E., Williams E. C., Conneally P. M., Benson M. D. Identification of a new hereditary amyloidosis prealbumin variant, Tyr-77, and detection of the gene by DNA analysis. J Clin Invest. 1988 Jan;81(1):189–193. doi: 10.1172/JCI113293. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Westermark P., Sletten K., Olofsson B. O. Prealbumin variants in the amyloid fibrils of Swedish familial amyloidotic polyneuropathy. Clin Exp Immunol. 1987 Sep;69(3):695–701. [PMC free article] [PubMed] [Google Scholar]
  29. Wright J. R., Calkins E. Amyloid in the aged heart: frequency and clinical significance. J Am Geriatr Soc. 1975 Mar;23(3):97–103. doi: 10.1111/j.1532-5415.1975.tb00968.x. [DOI] [PubMed] [Google Scholar]

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