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Updated: Jul 11, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Investigating the amyloidogenic nanostructured sequences of elastin: sequence encoded by exon 28 of human
Brigida Bochicchio1, Antonietta Pepe, Roberta Flamia
1Department of Chemistry, Università della Basilicata, Via N. Sauro 85, Potenza, Italy.
Abstract:
In this paper we demonstrate that the sequence encoded by exon 28 (EX28) of human tropoelastin gene is able to give amyloid-like fibrils. CD (circular dichroism) in solution and solid-state FTIR (Fourier transform infrared spectroscopy) spectroscopies have shown the presence of beta-sheet conformation. At the supramolecular level the fibers formed by EX28 peptide were investigated by AFM (atomic force microscopy) and ESEM (environmental scanning electron microscopy). A very big left-handed helix, 100 mum long, is visible together with aggregates of different sizes, some of them being constituted by helically interwoven fibers. Furthermore, an additional AFM image of EX28 is shown where the ultrastructure found is somewhat reminiscent of a more or less retiform film. These findings should be useful for designing proper elastin-inspired biomaterials.
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