Supplementary Material From Giant Axonal Neuropathy Alters the Structure of Keratin Intermediate Filaments in Human Hair

January 2017

Asfia Soomro, Richard J. Alsop, Atsuko Negishi, Laurent Kreplak, Douglas S. Fudge, Edward R. Kuczmarski, Robert D. Goldman, Maikel C. Rheinstädter

giant axonal neuropathy keratin intermediate filaments keratin tetramers X-ray diffraction tensile testing

TLDR Giant axonal neuropathy changes the structure of keratin in human hair, making it stiffer and stronger.

Giant axonal neuropathy (GAN) was found to alter the structure of keratin intermediate filaments in human hair. The study used X-ray diffraction and tensile testing to analyze hair from GAN patients, revealing a significant decrease in the 47 and 27 Å diffraction signals. Additionally, the hair was observed to be slightly stiffer, stronger, and more extensible. These findings suggested an increased positional disorder of the keratin tetramers within the hair fibers, indicating structural changes in the hair due to GAN.

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