Structural analyses of fibrinogen amyloid fibrils

Amyloid. 2007 Sep;14(3):199-203. doi: 10.1080/13506120701461111.

Abstract

Hereditary fibrinogen amyloidosis is characterized by deposition of amyloid fibrils in renal glomeruli. The subunit protein of the amyloid fibrils is a proteolytic fragment of the fibrinogen Aalpha-chain. To investigate the structure of fibrinogen amyloid, fibrils were extracted from the tissues of a patient and studied by X-ray fiber diffraction and electron microscopy. We have carried out a full structural characterization of amyloid fibrils taken from disease tissue. These studies revealed that ex vivo fibrinogen amyloid fibrils have a cross-beta structure similar to other chemical types of amyloid fibrils.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Amyloid / chemistry
  • Amyloid / ultrastructure*
  • Amyloidosis / pathology
  • Fibrinogen / chemistry
  • Fibrinogen / ultrastructure*
  • Humans
  • Protein Structure, Secondary*
  • Spleen / chemistry
  • Spleen / pathology
  • X-Ray Diffraction

Substances

  • Amyloid
  • fibrinogen Aalpha
  • Fibrinogen