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The local calcification inhibitor matrix Gla protein in pseudoxanthoma elasticum.

AbstractOBJECTIVES:
Recent studies have revealed the involvement of calcification inhibitory proteins in the pathogenesis of pseudoxanthoma elasticum (PXE).
DESIGN AND 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.
AuthorsDoris Hendig, Ralf Zarbock, Christiane Szliska, Knut Kleesiek, Christian Götting
JournalClinical biochemistry (Clin Biochem) Vol. 41 Issue 6 Pg. 407-12 (Apr 2008) ISSN: 0009-9120 [Print] United States
PMID18222176 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • ABCC6 protein, human
  • AHSG protein, human
  • Blood Proteins
  • Calcium-Binding Proteins
  • Extracellular Matrix Proteins
  • Multidrug Resistance-Associated Proteins
  • alpha-2-HS-Glycoprotein
  • matrix Gla protein
Topics
  • Adult
  • Aged
  • Blood Proteins (metabolism)
  • Calcinosis (metabolism, pathology)
  • Calcium-Binding Proteins (genetics, metabolism)
  • Extracellular Matrix Proteins (genetics, metabolism)
  • Female
  • Genotype
  • Haplotypes
  • Humans
  • Male
  • Middle Aged
  • Multidrug Resistance-Associated Proteins (genetics, metabolism)
  • Polymorphism, Genetic
  • Promoter Regions, Genetic
  • Pseudoxanthoma Elasticum (metabolism, pathology)
  • alpha-2-HS-Glycoprotein

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