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


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.
Götting
Christian
C
Institut für Laboratoriums- und Transfusionsmedizin, Herz- und Diabeteszentrum Nordrhein-Westfalen, Universitätsklinik der Ruhr-Universität Bochum, Bad Oeynhausen, Germany. cgoetting@hdz.nrw.de
Adam
Alexandra
A
Szliska
Christiane
C
Kleesiek
Knut
K
D000328:Adult; D019040:E-Selectin / Q000097:blood* / Q000235:genetics; D005260:Female; D005787:Gene Frequency; D005838:Genotype; D006801:Humans; D019041:L-Selectin / Q000097:blood* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D027425:ATP-Binding Cassette, Sub-Family C Proteins / Q000235:genetics; D009154:Mutation; D019007:P-Selectin / Q000097:blood* / Q000235:genetics; D010641:Phenotype; D011561:Pseudoxanthoma Elasticum / Q000097:blood* / Q000235:genetics
D019040:E-Selectin; D019041:L-Selectin; D027425:ATP-Binding Cassette, Sub-Family C Proteins; D019007:P-Selectin; C102029:ABCC6 protein, human