................................................................................................................................III SVENSK SAMMANFATTNING...........................................................................................IV ACKNOWLEDGEMENTS ......................................................................................................V LIST OF APPENDED PAPERS.........

.............................................................................................................................. III ACKNOWLEDGMENTS ........................................................................................................... V TABLE OF CONTENTS .......................................................................................................... VII LIST OF FIG...

High-resolution microscopic magnetic resonance imaging (μMRI) and diffusion tensor imaging (DTI) were performed to characterize brain structural abnormalities in a mouse model of mucopolysaccharidosis type VII (MPS VII). Microscopic magnetic resonance imaging demonstrated a decrease in the volume of anterior commissure and corpus callosum and a slight increase in the volume of the hippocampus i...

We have studied factor VII activation by measuring the ratio of factor VII clotting to coupled amidolytic activity (VIIc/VIIam) and cleavage of 125I-factor VII. In purified systems, a low concentration of Xa or a higher concentration of IXa rapidly activated 125I-factor VII, yielding a VIIc/VIIam ratio of 25 and similar gel profiles of heavy and light chain peaks of VIIa. On further incubation,...

........................................................................................................................................................ V ACKNOWLEDGEMENT............................................................................................................................... VII PUBLICATIONS .....................................................................................

A family with a peculiar defect of factor VII is described. The propositi, a brother and sister, were born of a nonconsanguineous marriage and presented a mild to moderate bleeding tendency since childhood (epistaxis, excessive bleeding after tooth extraction). The coagulation pattern was characterized by a mildly prolonged prothrombin time, normal partial thromboplastin time, and normal Stypve...

1. 0. G. Raabe, S. A. Book, N. J. Parks, Science 208, 61 (1980). 2. J. D. Kalbfleisch and R. L. Prentice, Statistical Analysis of Failure Time Data (Wiley, New York, 1980). 3. See for example, the absolute risk model [National Research Council, "Effects on populations of exposure to low levels of ionizing radiation" (BEIR Report PB 239, Washington, D.C., 1972), p. 171] and the recent analyses [...