What is it about?
Hair has an unusual fatty acid on its surface and in between the cuticle layers. It provides a method of allowing cuticles to naturally break away after mechanical insult to leave a fresh layer with "non-stick" properties. Maple Syrup Urine Disease (MSUD) is a rare condition where there is a deficiency in a particular enzyme that prevents branched-chain amino acids being metabolised. Consequently, isoleucine cannot be used by the body to produce this specific unusual fatty acid (18-methyleicosanoic acid, 18-MEA). Therefore, the natural cleavage plane within the cuticle structure is blocked. Hair surfaces from 10 MSUD patients have been imaged by atomic force microscopy (AFM) and cuticle breakage patterns discussed.
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Why is it important?
The paper provides further support for the importance of 18-MEA in hair science.
Read the Original
This page is a summary of: Maple syrup urine disease hair reveals the importance of 18-methyleicosanoic acid in cuticular delamination, Micron, April 2005, Elsevier, DOI: 10.1016/j.micron.2004.11.004.
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