Journal
PULMONARY CIRCULATION
Volume 3, Issue 4, Pages 851-855Publisher
SAGE PUBLICATIONS INC
DOI: 10.1086/674763
Keywords
pulmonary arterial hypertension (PAH); sickle cell disease (SCD); ubiquitin-proteasome pathway (UPP); apolipoprotein A (Apo-A)
Funding
- Empire Clinical Research Investigator Program grant
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Pulmonary arterial hypertension (PAH) is a major complication of sickle cell disease (SCD). Low levels of apolipoprotein A1 (Apo-A1) have been implicated in the development of PAH in SCD. We speculate that lower levels of Apo-A1 are related to dysregulation of the ubiquitin-proteasome pathway (UPP). Of 36 recruited patients with SCD, 14 were found to have PAH on the basis of right heart catheterization. Levels of Apo-A1 and Apo-B, polyubiquitin, total protease, and specific and normalized activity of chymotrypsin-like, trypsin-like, and caspase-like proteases in plasma were measured. Levels of Apo-A1 were found to be lower and polyubiquitin levels were found to be significantly higher in the PAH group (P < 0.05) in SCD. Apo-A levels were inversely correlated with polyubiquitin levels (r = -0.499, P = 0.009). These results indicate that lower levels of Apo-A1 in SCD patients with PAH are likely related to enhance degradation by UPP, potentially contributing to pulmonary vascular pathology. These findings may provide significant insight in identifying suitable therapeutic targets in these patients.
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