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Previous Volume 349:583-596 August 7, 2003 Number 6 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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The amyloidoses constitute a large group of diseases in which misfolding of extracellular protein has a prominent role. This dynamic process, which occurs in parallel with or as an alternative to physiologic folding, generates insoluble, toxic protein aggregates that are deposited in tissues in bundles of -sheet fibrillar protein (Figure 1). (A -sheet consists of strands of polypeptides in zigzag formation, as shown in Figure 2.) These amyloid deposits are identified on the basis of their apple-green birefringence under a polarized light microscope after staining with Congo red and the presence of rigid, nonbranching fibrils 7.5 to . . . [Full Text of this Article]

Molecular Mechanisms

Biochemical Characteristics of Amyloidogenic Proteins

A Dynamic View of the Pathogenic Process

Mutations and the Molecular Mechanism of Amyloid Formation

            Immunoglobulin Light Chains

            Familial Amyloidosis

            The Role of Instability

            Proteolysis

Amyloidosis as a Conformational Disease

Is Amyloidosis a Protein-Mediated Transmissible Disease?

The Common Constituents of Amyloid

Clinical Implications

Tissue Specificity of Amyloid Deposition

Mechanism of Tissue Damage

Diagnostic Problems and Pitfalls

Molecular Targets and Therapeutic Strategies

Effective Therapies

Future Perspectives

Source Information

From the Amyloid Center, Biotechnology Research Laboratory, University Hospital IRCCS Policlinico San Matteo; and the Department of Biochemistry, University of Pavia — both in Pavia, Italy.

Address reprint requests to Dr. Merlini at the Amyloid Center, Biotechnology Research Laboratory, Padiglione Forlanini, University Hospital IRCCS Policlinico San Matteo, Piazzale Golgi, 2, 27100 Pavia, Italy, or at gmerlini@smatteo.pv.it.

Related Letters:

Molecular Mechanisms of Amyloidosis
Sungur C. I., van der Hilst J. C.H., Simon A., Drenth J. P.H., Merlini G., Bellotti V.
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N Engl J Med 2003; 349:1872-1873, Nov 6, 2003. Correspondence

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