Pathophysiology of sickle cell disease
Since the discovery of sickle cell disease (SCD) in 1910, enormous strides have been made
in the elucidation of the pathogenesis of its protean complications, which has inspired recent …
in the elucidation of the pathogenesis of its protean complications, which has inspired recent …
Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology
Sickle cell disease (SCD) is a severe genetic blood disorder characterized by hemolytic
anemia, episodic vaso-occlusion, and progressive organ damage. Current management of …
anemia, episodic vaso-occlusion, and progressive organ damage. Current management of …
The gut microbiome regulates psychological-stress-induced inflammation
Psychological stress has adverse effects on various human diseases, including those of the
cardiovascular system. However, the mechanisms by which stress influences disease …
cardiovascular system. However, the mechanisms by which stress influences disease …
Therapeutic strategies for sickle cell disease: towards a multi-agent approach
For over 100 years, clinicians and scientists have been unravelling the consequences of the
A to T substitution in the β-globin gene that produces haemoglobin S, which leads to the …
A to T substitution in the β-globin gene that produces haemoglobin S, which leads to the …
Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease
Sickle cell disease (SCD) is characterized by recurring episodes of vascular occlusion in
which neutrophil activation plays a major role. The disease is associated with chronic …
which neutrophil activation plays a major role. The disease is associated with chronic …
Heme on innate immunity and inflammation
Heme is an essential molecule expressed ubiquitously all through our tissues. Heme plays
major functions in cellular physiology and metabolism as the prosthetic group of diverse …
major functions in cellular physiology and metabolism as the prosthetic group of diverse …
Cas9-AAV6 gene correction of beta-globin in autologous HSCs improves sickle cell disease erythropoiesis in mice
Abstract CRISPR/Cas9-mediated beta-globin (HBB) gene correction of sickle cell disease
(SCD) patient-derived hematopoietic stem cells (HSCs) in combination with autologous …
(SCD) patient-derived hematopoietic stem cells (HSCs) in combination with autologous …
Correction of sickle cell disease in transgenic mouse models by gene therapy
R Pawliuk, KA Westerman, ME Fabry, E Payen… - Science, 2001 - science.org
Sickle cell disease (SCD) is caused by a single point mutation in the human βA globin gene
that results in the formation of an abnormal hemoglobin [HbS (α2βS 2)]. We designed a βA …
that results in the formation of an abnormal hemoglobin [HbS (α2βS 2)]. We designed a βA …
Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing
Persistence of human fetal hemoglobin (HbF, α2γ2) in adults lessens the severity of sickle
cell disease (SCD) and the β-thalassemias. Here, we show that the repressor BCL11A is …
cell disease (SCD) and the β-thalassemias. Here, we show that the repressor BCL11A is …
Primary role for adherent leukocytes in sickle cell vascular occlusion: a new paradigm
Vascular occlusion is the major cause of morbidity and mortality in sickle cell disease but its
mechanisms are poorly understood. We demonstrate by using intravital microscopy in mice …
mechanisms are poorly understood. We demonstrate by using intravital microscopy in mice …