Send to

Choose Destination
See comment in PubMed Commons below
Biochim Biophys Acta. 2014 Mar;1841(3):353-61. doi: 10.1016/j.bbalip.2013.11.009. Epub 2013 Nov 27.

Role of cholesterol sulfate in epidermal structure and function: lessons from X-linked ichthyosis.

Author information

  • 1Dermatology Service, Department of Veterans Affairs Medical Center, and Department of Dermatology, University of California, San Francisco, CA USA. Electronic address:
  • 2Departments of Dermatology and Pediatrics, University of California, San Francisco, CA USA.
  • 3Department of Dermatology, Yonsei University, Wonju College of Medicine, Wonju, South Korea.
  • 4Medical Service, Department of Veterans Affairs Medical Center, and Department of Medicine, University of California, San Francisco, CA, USA.


X-linked ichthyosis is a relatively common syndromic form of ichthyosis most often due to deletions in the gene encoding the microsomal enzyme, steroid sulfatase, located on the short area of the X chromosome. Syndromic features are mild or unapparent unless contiguous genes are affected. In normal epidermis, cholesterol sulfate is generated by cholesterol sulfotransferase (SULT2B1b), but desulfated in the outer epidermis, together forming a 'cholesterol sulfate cycle' that potently regulates epidermal differentiation, barrier function and desquamation. In XLI, cholesterol sulfate levels my exceed 10% of total lipid mass (≈1% of total weight). Multiple cellular and biochemical processes contribute to the pathogenesis of the barrier abnormality and scaling phenotype in XLI. This article is part of a Special Issue entitled The Important Role of Lipids in the Epidermis and their Role in the Formation and Maintenance of the Cutaneous Barrier. Guest Editors: Kenneth R. Feingold and Peter Elias.


Cholesterol sulfate; Corneodesmosomes; Epidermal barrier function; Epidermal lipid metabolism; Steroid sulfatase; X-linked ichthyosis

[PubMed - indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science Icon for PubMed Central
    Loading ...
    Support Center