Pathophysiology of sickle cell disease

P Sundd, MT Gladwin, EM Novelli - Annual review of pathology …, 2019 - annualreviews.org
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 …

Sickle cell anemia, the first molecular disease: overview of molecular etiology, pathophysiology, and therapeutic approaches

MH Steinberg - The Scientific World Journal, 2008 - Wiley Online Library
The root cause of sickle cell disease is a single β‐globin gene mutation coding for the sickle
β‐hemoglobin chain. Sickle hemoglobin tetramers polymerize when deoxygenated …

Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology

D Zhang, C Xu, D Manwani… - Blood, The Journal of the …, 2016 - ashpublications.org
Sickle cell disease (SCD) is a severe genetic blood disorder characterized by hemolytic
anemia, episodic vaso-occlusion, and progressive organ damage. Current management of …

Insight into the complex pathophysiology of sickle cell anaemia and possible treatment

A Piccin, C Murphy, E Eakins… - European journal of …, 2019 - Wiley Online Library
Sickle cell anaemia (SCA) is the consequence of abnormal haemoglobin production due to
an inherited point mutation in the β‐globin gene. The resulting haemoglobin tetramer is …

Pathophysiologically based drug treatment of sickle cell disease

MH Steinberg - Trends in Pharmacological Sciences, 2006 - cell.com
Sickle cell disease is a systemic disorder that is caused by a mutation (Glu6Val) in the gene
that encodes β globin. The sickle hemoglobin molecule (HbS) is a tetramer of two α-globin …

[HTML][HTML] Vasculopathy in sickle cell disease: biology, pathophysiology, genetics, translational medicine and new research directions

GJ Kato, RP Hebbel, MH Steinberg… - American journal of …, 2009 - ncbi.nlm.nih.gov
Sickle cell disease has been very well characterized as a single amino acid molecular
disorder of hemoglobin leading to its pathological polymerization, with resulting red cell …

The intriguing contribution of white blood cells to sickle cell disease–a red cell disorder

I Okpala - Blood reviews, 2004 - Elsevier
Sickle cell disease (SCD) is characterized by a point mutation that replaces adenine with
thymidine in the sixth codon of the β-globin gene, a unique morphological abnormality of red …

[HTML][HTML] Ischemia-reperfusion injury in sickle cell disease: from basics to therapeutics

J Ansari, FNE Gavins - The American Journal of Pathology, 2019 - Elsevier
Sickle cell disease (SCD) is one of the most common hereditary hemoglobinopathies
worldwide, affecting almost 400,000 newborns globally each year. It is characterized by …

Pathophysiology and therapy for haemoglobinopathies; Part I: sickle cell disease

C Madigan, P Malik - Expert reviews in molecular medicine, 2006 - cambridge.org
In sickle cell disease, a single base pair substitution in the gene encoding the β-globin chain
of the haemoglobin molecule gives rise to a surprisingly broad spectrum of …

The red blood cell—inflammation vicious circle in sickle cell disease

E Nader, M Romana, P Connes - Frontiers in immunology, 2020 - frontiersin.org
Sickle cell disease (SCD) is a genetic disease caused by a single mutation in the β-globin
gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS) …