Background: Hemophilia B is a bleeding disorder with a recessive X-linked inheritance pattern, in which the infected individuals have low levels of factor IX in their plasma. Affected individuals may have bleeding episodes after trauma or spontaneously considering the plasma level of factor IX. In order to prevent these episodes and to control bleeding, they should use coagulation factor concen...

To investigate the temporal and topographic changes in the expression of various collagens during the process of endochondral bone formation, qualitative and quantitative analysis of the collagens synthesized by organ cultures from the separated presumptive calcification and permanent cartilaginous regions of embryonic chick sternum at various stages of development was performed. Special emphas...

Several studies describing the ultrastructure and extracellular matrix (ECM) of intervertebral discs (IVDs) involve animal models and specimens obtained from symptomatic individuals during surgery for degenerative disease or scoliosis, which may not necessarily correlate to changes secondary to normal aging in humans. These changes may also be segment-specific based on different load patterns t...

OBJECTIVE The platelet glycoprotein (GP)Ib-V-IX complex is a receptor required for normal hemostasis deficient in the Bernard-Soulier bleeding disorder. To evaluate the consequences of GPIb-V-IX deficiency in thrombosis we generated mouse models of the disease by targeting the GPIb beta subunit. METHODS AND RESULTS Complete deletion (GPIb beta-/-) or an intracellular truncation (GPIb beta del...

Previously undescribed disulfide-bonded collagenous pepsin-derived fragments have been isolated from fetal calf tendon and skin. One fragment, 10.5 kDa after reduction, was shown to be similar but distinct to the COL1 domain of the recently characterized type XII collagen (64% primary structure identity). The similarity includes important features such as size, location of the cysteine residues...

Bostjan Kobe THE UNIVERSITY OF QUEENSLAND Understanding the mechanism of platelet activation requires the knowledge of the structural organization of the glycoprotein (GP) Ib-IX-V receptor. In this issue of Blood, an innovative structural approach was used to shed light on the interaction between GPIb and GPIX subunits.1 The GPIb-IX-V complex serves as the platelet receptor that mediates bindin...

A new molecule, type XIV collagen, with domains homologous to type IX and XII collagens has been recently discovered in pepsin extracts of fetal bovine tissues (Dublet, B., and van der Rest, M. (1991) J. Biol. Chem. 266, 6853-6858). In the present study, we describe the purification and the characterization of the intact native form of this newly discovered collagen. By using only two chromatog...

in this study, anion-exchange chromatography was used to purify factor vii and factor ix from prothrombin complex (ppsb), which contains coagulation factors ii, vii, ix and x. for this purpose, deae-sepharose cl-6b gel , pharmacia column xk-26 , high flow rate and two stepwise gradients with phosphate citrate buffer were used. the yield of the two lyophylized products, factor vii and factor ix ...

Immortalized human chondrocytes were established by transfection of primary cultures of juvenile costal chondrocytes with vectors encoding simian virus 40 large T antigen and selection in suspension culture over agarose. Stable cell lines were generated that exhibited chondrocyte morphology, continuous proliferative capacity (> 80 passages) in monolayer culture in serum-containing medium, and e...