Two different types of amyloid deposits - Apolipoprotein A-IV and transthyretin - In a patient with systematic amyloidosis

Joakim Bergström, Charles L. Murphy, Deborah T. Weiss, Alan Solomon, Knut Sletten, Ulf Hellman, Per Westermark

Research output: Contribution to journalArticle

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Abstract

Certain forms of systemic arnyloidosis have been associated with the pathologic deposition as fibrils of three different apolipoprotein-related proteins-apolipoprotein A-I, apolipoprotein A-II, and serum arnyloid A. We have previously reported (Bergström et al, Biochem Biophys Res Commun 2001;285:903-908) that arnyloid fibrils extracted from the heart of an elderly male with senile systemic arnyloidosis contained, in addition to wild-type transthyretin-related molecules, an N-terminal fragment of yet a fourth apolipoprotein-apolipoprotein A-IV (apoA-IV). We now provide the results of our studies that have established the complete amino-acid sequence of this ∼70-residue component and, additionally, have shown this protein to be the product of an unmutated apoA-IV gene. Notably, the apoA-IV and transthyretin fibrils were not codeposited but, rather, had anatomically distinct patterns of distribution within the heart and other organs, as evidenced immunohistochemically, by variation in the ultra structural morphology and by differences in the intensity of Congo red birefringence. These findings provide the first conclusive evidence that two separate forms of arnyloid, each derived from a wild-type arnyloidogenic precursor protein, were present in a patient with systemic arnyloidosis.

Original languageEnglish (US)
Pages (from-to)981-988
Number of pages8
JournalLaboratory Investigation
Volume84
Issue number8
DOIs
StatePublished - Aug 1 2004

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Prealbumin
Amyloid Plaques
Amyloidosis
Apolipoproteins
Apolipoprotein A-II
Birefringence
Congo Red
Protein Precursors
Apolipoprotein A-I
Amino Acid Sequence
Proteins
Serum
Genes
apolipoprotein A-IV

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology

Cite this

Two different types of amyloid deposits - Apolipoprotein A-IV and transthyretin - In a patient with systematic amyloidosis. / Bergström, Joakim; Murphy, Charles L.; Weiss, Deborah T.; Solomon, Alan; Sletten, Knut; Hellman, Ulf; Westermark, Per.

In: Laboratory Investigation, Vol. 84, No. 8, 01.08.2004, p. 981-988.

Research output: Contribution to journalArticle

Bergström, Joakim ; Murphy, Charles L. ; Weiss, Deborah T. ; Solomon, Alan ; Sletten, Knut ; Hellman, Ulf ; Westermark, Per. / Two different types of amyloid deposits - Apolipoprotein A-IV and transthyretin - In a patient with systematic amyloidosis. In: Laboratory Investigation. 2004 ; Vol. 84, No. 8. pp. 981-988.
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