- Molecular Mechanisms of Hepatic Dysfunction in Sickle Cell Disease: Lessons From The Townes Mouse Modelby Tirthadipa Pradhan-Sundd on June 27, 2022 at 10:00 am
Sickle cell disease (SCD) is an autosomal-recessive-genetic disorder that affects ~100,000 Americans and millions of people worldwide. Erythrocyte sickling, vaso-occlusion, sterile inflammation and hemolysis are the major pathophysiological pathways leading to liver injury in SCD. Although hepatic dysfunction affects up to 10-40% of SCD patients, therapeutic approaches to prevent liver injury in SCD are not known, and the molecular mechanisms promoting progressive liver injury in SCD remain...
- Comprehensive assessment of cognitive function in adults with moderate and severe sickle cell diseaseby Gerard T Portela on June 24, 2022 at 10:00 am
- Liver to lung microembolic NETs promote Gasdermin-D-dependent inflammatory lung injury in Sickle Cell Diseaseby Ravi Vats on June 23, 2022 at 10:00 am
Acute lung injury, referred to as the acute chest syndrome, is a major cause of morbidity and mortality in patients with sickle cell disease (SCD), which often occurs in the setting of a vaso-occlusive painful crisis. P-selectin antibody therapy reduces hospitalization of SCD patients by almost 50%, suggesting that an unknown P-selectin-independent mechanism promotes remaining vaso-occlusive events. In SCD patients, intra-erythrocytic polymerization of mutant hemoglobin promotes...
- Nrf2 Activation With CDDO-Methyl Promotes Beneficial and Deleterious Clinical Effects in Transgenic Mice With Sickle Cell Anemiaby Chibueze A Ihunnah on May 27, 2022 at 10:00 am
Activation of Nrf2, a major transcription factor that drives the antioxidant defense system, is an emerging therapeutic strategy in Sickle Cell Disease (SCD). In this study, transgenic Sickle Cell Anemia mice (SS mice) treated with CDDO-Methyl (CDDO-Me), a potent Nrf2 activator, showed reduced progression of hemolytic anemia with aging, but surprisingly also showed reduced endothelial function. Pulmonary vessels isolated from SS mice treated for 4 months with CDDO-Me displayed a diminished...
- Revisiting Arginine Therapy for Sickle Cell Acute Vaso-occlusive Painful Crisisby Rachel K Hopper on May 13, 2022 at 10:00 am