Supplementary Material From Giant Axonal Neuropathy Alters the Structure of Keratin Intermediate Filaments in Human Hair
January 2017
Studysummary This study found that keratin intermediate filaments in the hair of patients with giant axonal neuropathy were structurally altered, resulting in hair that was stiffer, stronger, and more extensible.
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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.
