Hemophilia B is a leading target for gene therapy because current therapy is not optimal. Hence, a murine model of factor IX (F. IX) deficiency was generated to develop gene therapy strategies for hemophilia B. A targeting vector was created by replacing a 3.2-kb segment of the gene encompassing the catalytic domain with a phosphoglycerokinase promoter-driven neomycin resistant (neor) gene cass...

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We model dependencies between m multivariate continuous-valued information sources by a combination of (i) a generalized canonical correlations analysis (gCCA) to reduce dimensionality while preserving dependencies in m − 1 of them, and (ii) summarizing dependencies with the remaining one by associative clustering. This new combination of methods avoids multiway associative clustering which wou...

We describe the synthesis of a haemoglobin (Hb) wrapped covalently by recombinant human serum albumin mutants [HSA(Y161H)] containing Mn(III) protoporphyrin IX (MnPP), the Hb-[HSA(Y161H)-MnPP]3 cluster, highlighting the formation of its O2-complex stable even in H2O2 solution.

The binding of porphyrins by two serum proteins, hemopexin and albumin, has been compared using a fluorescence method in order to clarify the biological roles of the proteins in the disposal of these pigments. The affinity of hemopexin for metalloporphyrins increases in the order: nickel-deuteroporphyrin IX < cobalt-deuteroporphyrin IX < iron-deuteroporphyrin IX (deuteroheme) N iron-protoporphy...

The matrilins are a family of four noncollagenous oligomeric extracellular matrix proteins with a modular structure. Matrilins can act as adapters which bridge different macromolecular networks. We therefore investigated the effect of collagen IX deficiency on matrilin-3 integration into cartilage tissues. Mice harboring a deleted Col9a1 gene lack synthesis of a functional protein and produce c...

Current antithrombotic drugs have an adverse effect on bleeding, highlighting the need for new molecular targets for developing antithrombotic drugs that minimally affect hemostasis. Here we show that LIMK1(-/-) mice have defective arterial thrombosis in vivo but do not differ from wild-type mice with respect to bleeding time. LIMK1(-/-) mice show a selective defect in platelet activation induc...

In aiming to develop a gene therapy approach for hemophilia B, we expressed and characterized human factor IX in rat capillary endothelial cells (CECs). Moloney murine leukemia virus-derived retrovirus vectors that contain human factor IX cDNA linked to heterologous promoters and the neomycin-resistant gene were constructed and employed to prepare recombinant retroviruses. Rat CECs and NIH 3T3 ...