title: The local calcification inhibitor matrix Gla protein in pseudoxanthoma elasticum.
abstract: OBJECTIVE
  Recent studies have revealed the involvement of calcification inhibitory proteins in the pathogenesis of pseudoxanthoma elasticum (PXE).
  
  
  METHODS
  We analyzed serum concentrations of the calcification inhibitor matrix Gla protein (MGP) in a large cohort of patients suffering from PXE (n=101), 34 first-degree relatives and 67 healthy controls. Moreover, we determined the distribution of the two MGP promoter polymorphisms c.-7G>A and c.-138T>C in the three cohorts.
  
  
  RESULTS
  We found significantly lower total MGP concentrations in the sera of PXE patients compared to healthy controls (p=0.0002). Furthermore, higher serum MGP concentrations could be correlated with a later PXE onset. Analysis of MGP promoter polymorphism frequencies revealed one MGP haplotype to be a potential protective co-factor in PXE.
  
  
  CONCLUSIONS
  Our findings point to a role of the local calcification inhibitor MGP in PXE manifestation.
authors.affiliation: Institut für Laboratoriums- und Transfusionsmedizin, Herz- und Diabeteszentrum Nordrhein-Westfalen, Universitätsklinik der Ruhr-Universität Bochum, Bad Oeynhausen, Germany.
authors.forename: Doris
authors.initials: D
authors.lastname: Hendig
authors.forename: Ralf
authors.initials: R
authors.lastname: Zarbock
authors.forename: Christiane
authors.initials: C
authors.lastname: Szliska
authors.forename: Knut
authors.initials: K
authors.lastname: Kleesiek
authors.forename: Christian
authors.initials: C
authors.lastname: Götting
mesh_terms: D000328:Adult; D000368:Aged; D001798:Blood Proteins / Q000378:metabolism; D002114:Calcinosis / Q000378:metabolism / Q000473:pathology; D002135:Calcium-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000378:metabolism*; D005260:Female; D005838:Genotype; D006239:Haplotypes; D006801:Humans; D008297:Male; D008875:Middle Aged; D027425:ATP-Binding Cassette, Sub-Family C Proteins / Q000235:genetics / Q000378:metabolism; D011110:Polymorphism, Genetic; D011401:Promoter Regions, Genetic; D011561:Pseudoxanthoma Elasticum / Q000378:metabolism / Q000473:pathology; D060749:alpha-2-HS-Glycoprotein; D000097568:Matrix Gla Protein
chemical_list: D001798:Blood Proteins; D002135:Calcium-Binding Proteins; D016326:Extracellular Matrix Proteins; D027425:ATP-Binding Cassette, Sub-Family C Proteins; D060749:alpha-2-HS-Glycoprotein; D000097568:Matrix Gla Protein; C102029:ABCC6 protein, human; C556354:AHSG protein, human