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

Peter M. Elias, Mary L. Williams, Eung Ho Choi, Kenneth R. Feingold

Research output: Contribution to journalReview article

49 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)353-361
Number of pages9
JournalBiochimica et Biophysica Acta - Molecular and Cell Biology of Lipids
Volume1841
Issue number3
DOIs
Publication statusPublished - 2014 Mar 1

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X-Linked Ichthyosis
Epidermis
Biochemical Phenomena
Steryl-Sulfatase
Ichthyosis
Lipids
Gene Deletion
X Chromosome
Maintenance
Phenotype
Weights and Measures
Skin
Enzymes
Genes
cholesteryl sulfate

All Science Journal Classification (ASJC) codes

  • Molecular Biology
  • Cell Biology

Cite this

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abstract = "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.",
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Role of cholesterol sulfate in epidermal structure and function : Lessons from X-linked ichthyosis. / Elias, Peter M.; Williams, Mary L.; Choi, Eung Ho; Feingold, Kenneth R.

In: Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids, Vol. 1841, No. 3, 01.03.2014, p. 353-361.

Research output: Contribution to journalReview article

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