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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.

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

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