Circulating P-, L- and E-selectins in pseudoxanthoma elasticum patients.

Abstract:

OBJECTIVES:Pseudoxanthoma elasticum (PXE) is a hereditary disorder predominantly affecting the skin, retina and vascular system. The aim of this study was to measure cell adhesion molecules in PXE patients. DESIGN AND METHODS:Soluble P-, E- and L-selectins were measured in 61 non-consanguineous PXE patients. The distribution of the variants E-selectin S128R and P-selectin T715R were determined. RESULTS:P-selectin concentrations were significantly increased in male and female PXE patients. Furthermore, P-selectin levels correlated with the ABCC6 gene status of the PXE patients. Patients harboring two mutant ABCC6 alleles had 1.5-fold increased P-selectin concentrations in comparison to patients with at least one wild-type allele. E- and L-selectin levels were within normal range and the allelic frequencies of the investigated polymorphisms did not differ between patients and age- and sex-matched controls. CONCLUSIONS:Our data show elevated P-selectin levels in PXE patients potentially due to oxidative stress and elevated protease activity in PXE.

journal_name

Clin Biochem

journal_title

Clinical biochemistry

authors

Götting C,Adam A,Szliska C,Kleesiek K

doi

10.1016/j.clinbiochem.2007.12.009

subject

Has Abstract

pub_date

2008-04-01 00:00:00

pages

368-74

issue

6

eissn

0009-9120

issn

1873-2933

pii

S0009-9120(07)00489-4

journal_volume

41

pub_type

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