H. Krebs, W. Schramm (auth.), Professor Dr.med. Inge's 36th Hemophilia Symposium Hamburg 2005 PDF

By H. Krebs, W. Schramm (auth.), Professor Dr.med. Inge Scharrer, Professor Dr.med. Wolfgang Schramm (eds.)

This publication includes the contribution to the thirty sixth Hemophilia Symposium, Hamburg 2005. the most issues are epidemiolgy, hemophilia remedy, orthopedic remedy in hemophiliacs, hemostaseologic analysis and pediatric hemostaseology. the amount is rounded off by means of various unfastened papers and posters on hemophilia, inhibitors in hemophilia and diagnostics.

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5%) is given in Table 2. Compared to the data of previous surveys these are relative consistent findings. e. 9%) as shown in Table 2. 7% of patients with von Willebrand disease showed Ristocetin cofactor levels below 30% as demonstrated in Table 2. Table 2. 9% (19) of the patients with hemophilia B an inhibitor was found (see Table 2). These findings correspond to international large-scale prevalence studies and registry data indicating that the prevalence of inhibitors in the hemophiliac A population overall is up to 5% [6].

Neels JG, Bovenschen N, Zonneveld A-J, Lenting P. Interaction between factor VIII and LDL receptor-related protein. Trends Cardiovasc Med 2000; 10: 8–14. 33. Herz J, Strickland DK. LRP: a multifunctional scavenger and signaling receptor. J Clin Invest 2001; 108: 779–84. 34. Neels JG, Berg BMM, Looken A, Olivecrona G, Pannekoek H, Zonneveld A-J. The second and fourth cluster of class A cysteine-rich repeats of the low density lipoprotein receptorrelated protein share ligand-binding properties. J Biol Chem 1999; 274: 31305–11.

Other domains present in all receptors include epidermal-growth factor (EGF)-like repeats and YWTD b-propeller domains. 8 nM) as studied by SPR [34]. Interestingly, the A3 region 1811-1818 of LCh is positioned in close proximity to the membrane surface [22] so that a slightly higher affinity of LCh to cluster IV is consistent with location of cluster IV closest to the cell surface. Our group studied interaction of isolated A2 domain of HCh with recombinant clusters II, III and IV. We demonstrated that A2 was also able to bind to immobilized cluster II and cluster IV (Kd 20-50 nM) in a RAP-sensitive manner in SPR-based assay, but not to cluster III [35].

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