6 Publication(s) found
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Hematology, Hemophilia
10. Jan 2018

The endothelial specific isoform of type XVIII collagen correlates to annual bleeding rate in haemophilia patients

The medical need in the haemophilic (HF) field to reduce bleeding incidents requires measurement of the annual bleeding rate (ABR) in haemophiliacs. Vascular rupture is associated with damage to the vascular endothelium causing exposure of the basement membrane. Endothelial cells and …

Hematology, Hemophilia
29. Aug 2017

Increased basement membrane turnover and strongly attenuated interstitial matrix turnover is a key pathological feature of haemophilia

Altered extracellular matrix (ECM) turnover is a central pathological feature of many diseases. The ECM is a meshwork of molecules that provides structural support to cells throughout the body. It consists mainly of two membranes: the interstitial matrix, which is present between parenchymal cells i…

Rheumatology, Hematology, Hemophilia
24. Apr 2017

Serological biomarkers detect active joint destruction and inflammation in patients with haemophilic arthropathy

Progressive arthropathy caused by recurrent joint bleeds is a severe complication in haemophilia.

We investigated whether biomarkers of cartilage and bone degradation, and inflammation were altered in haemophilia patients and whether these biomarkers could identify haemophili…

Hematology, Hemophilia
28. Oct 2016

Altered collagen turnover in factor VIII-deficient rats with hemophilic arthropathy identifies potential novel serological biomarkers in hemophilia

Joint bleeding in hemophilia may induce significant remodeling of the extracellular matrix. Biomarkers of collagen turnover were investigated in a F8-/- rat model of hemophilic arthropathy. Biomarkers of cartilage degradation increased significantly during development of arthropathy. B…

22. Oct 2011

Assessment of proteolytic degradation of the basement membrane: a fragment of type IV collagen as a biochemical marker for liver fibrosis

Collagen deposition and an altered matrix metalloproteinase (MMP) expression profile are hallmarks of fibrosis. Type IV collagen is the most abundant structural basement membrane component of tissue, which increases 14-fold during fibrogenesis in the liver. Proteolytic degradation of co…