2013
DOI: 10.1086/674754
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Cellular, Pharmacological, and Biophysical Evaluation of Explanted Lungs from a Patient with Sickle Cell Disease and Severe Pulmonary Arterial Hypertension

Abstract: Pulmonary hypertension is recognized as a leading cause of morbidity and mortality in patients with sickle cell disease (SCD). We now report benchtop phenotyping from the explanted lungs of the first successful lung transplant in SCD. Pulmonary artery smooth muscle cells (PASMCs) cultured from the explanted lungs were analyzed for proliferate capacity, superoxide (O 2 • − ) production, and changes in key pulmonary arterial hypertension (PAH)-associated molecules and compared with non-PAH PASMCs. Upregulation o… Show more

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Cited by 25 publications
(17 citation statements)
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References 74 publications
(135 reference statements)
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“…NO destruction by hemoglobin can also cause further impairment in vascular endothelial function via activation of adhesion molecules and potent vasoconstrictors such as endothelin 1 60 . This phenomenon, foreshadowed by evidence of NO scavenging and oxidant stress with early generation blood substitutes, 80 has now been implicated as a mechanism of NO depletion in the RBC storage lesion 70 and other hemolytic conditions such as thalassemia, malaria, and paroxysmal nocturnal hemoglobinuria 67 , 81 83 …”
Section: Physiology Of Conditionally Essential Amino Acidsmentioning
confidence: 99%
“…NO destruction by hemoglobin can also cause further impairment in vascular endothelial function via activation of adhesion molecules and potent vasoconstrictors such as endothelin 1 60 . This phenomenon, foreshadowed by evidence of NO scavenging and oxidant stress with early generation blood substitutes, 80 has now been implicated as a mechanism of NO depletion in the RBC storage lesion 70 and other hemolytic conditions such as thalassemia, malaria, and paroxysmal nocturnal hemoglobinuria 67 , 81 83 …”
Section: Physiology Of Conditionally Essential Amino Acidsmentioning
confidence: 99%
“…22 Regional accumulations of medial and adventitial collagen also co-localize with decreases in PAC in sickle-cell associated PAH. 23 PASMCs in the media and fibroblasts in the vessel adventitia are generally identified as the cellular source for this regional collagen accumulation, though pulmonary arterial endothelial cells (PAECs) that trans-differentiate into PASMC-like cells likely also contribute. 24 …”
Section: Cellular and Molecular Mechanisms Responsible For Modulatingmentioning
confidence: 99%
“…TSP1 expression was increased in aged animals (Rogers et al 2014) and is associated with decreased tissue levels of cGMP (Isenberg et al 2007) and less cutaneous blood flow at baseline and after pharmacologic or thermal challenge (Rogers et al 2014). Ischemic myocutaneous flaps in senescent wild type mice demonstrated decreased blood flow and poor survival compared to younger mice (Isenberg et al 2007).…”
Section: Enhanced Tsp1-cd47 Signaling In Pre-clinical Models Of Cvdmentioning
confidence: 99%
“…Though rare in comparison to premature MI, these genetic variations in PAH implicate the importance of TSP1 in promoting pulmonary vascular pathology. Preliminary studies in lungs explanted from patients undergoing transplantation for end-stage PAH found significant upregulation of the TSP1-CD47 signaling axis at both the mRNA and protein level in whole lung (Bauer et al 2012) and in the proximal and distal isolated pulmonary artery vasculature (Rogers et al 2014). Intriguingly, a positive correlation with elevated plasma TSP1 levels and adverse outcomes, including death, in PAH has recently been described (http://scidok.sulb.uni-saarland.de/volltexte/2012/4692/pdf/Dissertation_Christian_Frantz_2011.pdf).…”
Section: The Cd47-tsp1 Signaling Axis In Human Cvdmentioning
confidence: 99%