Maple syrup urine disease hair reveals the importance of 18-methyleicosanoic acid in cuticular delamination

James R. Smith, J. Alan Swift
  • Micron, April 2005, Elsevier
  • DOI: 10.1016/j.micron.2004.11.004

Hair from patients with a rare genetic disease provide hair structural insights

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.

Why is it important?

The paper provides further support for the importance of 18-MEA in hair science.

Perspectives

Dr James R Smith (Author)
University of Portsmouth

AFM is a particularly valuable technique for imaging hair surfaces, combining high resolution and minimal sample preparation.

Read Publication

http://dx.doi.org/10.1016/j.micron.2004.11.004

The following have contributed to this page: Dr James R Smith