Differential Expression of Matrix Metalloproteinases in the Serum of Patients with Mucopolysaccharidoses View Full Text


Ontology type: schema:Chapter      Open Access: True


Chapter Info

DATE

2011-09-22

AUTHORS

Spyros P. Batzios , Dimitrios I. Zafeiriou , Euthymia Vargiami , George Karakiulakis , Eleni Papakonstantinou

ABSTRACT

Mucopolysaccharidoses (MPS) represent a heterogeneous group of hereditary disorders, characterized by accumulation of glycosaminoglycans within the lysosomes. The objective of this study was to elucidate the expression and activity of matrix metalloproteinases (MMPs) in the serum of pediatric patients with MPS. Serum gelatinase activity was assessed by gelatin zymography and the concentration of circulating MMP-2, MMP-9, and of tissue inhibitors of MMPs (TIMP)-1 and TIMP-2 was measured by ELISA in the serum of seven patients with MPS (five with MPS III, 1 with MPS II and 1 with MPS VI), and healthy age- and sex-matched participants. Serum activity and protein levels of MMP-9 were significantly reduced whereas of MMP-2 were significantly increased in patients with MPS III, as compared to controls. There were no significant alterations in serum protein levels of TIMP-1 and TIMP-2 in patients with MPS III, as compared to controls. In MPS II, proMMP-2 activity and protein levels of MMP-2 were significantly increased, as compared to control. In MPS VI, enzyme replacement therapy reduced the activity and protein levels of MMP-9 up to 4 months after the initiation of treatment. The reported alterations in the expression of MMPs in the serum of patients with MPS suggest that these molecules may be used as potential biomarkers for the diagnosis, follow-up and response to therapy in patients with MPS. More... »

PAGES

59-66

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/8904_2011_58

DOI

http://dx.doi.org/10.1007/8904_2011_58

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1050801452

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/23430874


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