PNH is a rare and severe blood disorder characterized by the destruction of red blood cells, known as intravascular hemolysis (IVH), and white blood cell and platelet activation that can cause thrombosis (blood clots) and result in organ damage and potentially premature death.1-3 Marc Dunoyer,...
a bleeding disorder arises when there is a deficiency of protein C inhibitor. Factor V is a single-chain glycoprotein of approximately 340 000 mol. wt., and it circulates at a concentration of about 7 μg/mL in the blood.26It is activated by thrombin to formfactor Va, which has two ch...
The following is heavily based on Trueblood et al., 1996 and references therein. Displacement of atoms from their mean position in a crystal structure diminishes the scattered X-ray intensity. The displacement may be the result of temperature-dependent atomic vibrations or static disorder in a cry...
Given its multiple homeostatic functions, we hypothesise that ERG may have an anti-thrombotic effect in EC, thus protecting blood vessels from spontaneous clot formation. In this study, we show that inducible chronic endothelial-specific deletion of ERG in adult mice leads to systemic coagulopathy wi...
Blood-device interaction Von Willebrand Factor (vWF) Von Willebrand factoris a large heterogeneous multimericglycoproteinwith molecular weight ranging up to >20,000kDa. Eachmonomerhas a molecular weight ranging between 250 and 270kDa and is primarily synthesized into multimers and constitutively secrete...
Structural Analysis of N-Linked Sugar Chains of Human Blood Clotting Factor IX The structures of N-glycans of human blood clotting factor IX were studied. N-Glycans liberated by hydrazinolysis were N-acetylated and the reducing-end su... Y Makino,K Omichi,N Kuraya,... - 《Journal of Bioc...
(i.e. like the first two events) this suggests that the first two events introduce disorder into the molecule (conformational changes) while the third and last event stabilizes the final molecule resulting from this disorder/conformational change, i.e., dissociation into a monomeric state. Our ...
Falls occurred in 36% of SDAT individuals versus 11% of controls. The higher frequency of falls in demented participants was not explained by greater neurologic deficit nor by increased drug use compared with controls. However, males with SDAT who reported falls, had higher mean blood pressures ...
Based on this ex vivo data we expected impaired red blood cell parameters for Grsf1−/− mice. This was, however, not the case. In fact, Grsf1−/− mice carry a fully functional erythropoietic system, which is indicated by the lacking differences in the major erythropoietic parameters...
FGFR-related fusion proteins which are caused by intragenic chromosomal translocation have been detected in hematological cancers (Table4). These fusion proteins are mainly found in patients diagnosed with stem cell leukemia lymphoma (SCLL) (also known as 8p11 myeloproliferative) syndrome. FGFR fusion ...