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

doi:10.1016/j.bbalip.2013.11.009

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

Dermatology

Research output: Contribution to journal › Review article › peer-review

97 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 language	English
Pages (from-to)	353-361
Number of pages	9
Journal	Biochimica et Biophysica Acta - Molecular and Cell Biology of Lipids
Volume	1841
Issue number	3
DOIs	https://doi.org/10.1016/j.bbalip.2013.11.009
Publication status	Published - 2014 Mar

Bibliographical note

Funding Information:
Ms. Joan Wakefield provided superb editing, organizational and graphics skills for this manuscript. This work was supported by NIH grant AR061106 , and by the Medical Research Service, Department of Veterans Affairs . These contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIAMS or NIH.

All Science Journal Classification (ASJC) codes

Molecular Biology
Cell Biology

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10.1016/j.bbalip.2013.11.009

Cite this

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

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.",

keywords = "Cholesterol sulfate, Corneodesmosomes, Epidermal barrier function, Epidermal lipid metabolism, Steroid sulfatase, X-linked ichthyosis",

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note = "Funding Information: Ms. Joan Wakefield provided superb editing, organizational and graphics skills for this manuscript. This work was supported by NIH grant AR061106 , and by the Medical Research Service, Department of Veterans Affairs . These contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIAMS or NIH.",

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AU - Williams, Mary L.

AU - Choi, Eung Ho

AU - Feingold, Kenneth R.

N1 - Funding Information: Ms. Joan Wakefield provided superb editing, organizational and graphics skills for this manuscript. This work was supported by NIH grant AR061106 , and by the Medical Research Service, Department of Veterans Affairs . These contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIAMS or NIH.

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N2 - 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.

AB - 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.

KW - Cholesterol sulfate

KW - Corneodesmosomes

KW - Epidermal barrier function

KW - Epidermal lipid metabolism

KW - Steroid sulfatase

KW - X-linked ichthyosis

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

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