Journalartikel

Jahr der Veröffentlichung: 2015

Seiten: 403-412

Zeitschrift: Journal of Lipid Research

Bandnummer: 56

Heftnummer: 2

ISSN: 0022-2275

eISSN: 1539-7262

Open Access Status: Hybrid

DOI Link: https://doi.org/10.1194/jlr.M055608

Verlag: Elsevier

Abstract: 
Steroid sulfatase (STS) deficiency is the underlying cause of the skin condition known as recessive X-linked ichthyosis (RXLI). RXLI patients show scales on their skin caused by high concentrations of cholesterol sulfate (CS), as they are not capable of releasing the sulfate group from its structure to obtain free cholesterol. CS has been reported, so far, as the sole sulfated steroid with increased concentrations in the blood of RXLI patients. A non-targeted LC-MS approach in negative mode detection (LC-MS precursor ion scan mode) was applied to serum samples of 12 RXLI patients and 19 healthy males. We found that CS was not the only sulfated compound consistently elevated in RXLI patients, because a group of compounds with a m/z of 481 was found in high concentrations too. Further LC-MS/MS demonstrated that the main contributor to the m/z 481 signal in RXLI serum is 27-hydroxycholesterol-3-sulfate (27OHC3S). Accordingly, a new method for 27OHC3S quantification in the context of RXLI has been developed and validated. Other hydroxycholesterol sulfate compounds were elevated as well in RXLI patients.

Harvard-Zitierstil: Sanchez-Guijo, A., Oji, V., Hartmann, M., Schuppe, H., Traupe, H. and Wudy, S. (2015) High levels of oxysterol sulfates in serum of patients with steroid sulfatase deficiency, Journal of Lipid Research, 56(2), pp. 403-412. https://doi.org/10.1194/jlr.M055608

APA-Zitierstil: Sanchez-Guijo, A., Oji, V., Hartmann, M., Schuppe, H., Traupe, H., & Wudy, S. (2015). High levels of oxysterol sulfates in serum of patients with steroid sulfatase deficiency. Journal of Lipid Research. 56(2), 403-412. https://doi.org/10.1194/jlr.M055608