Abstract:
: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 ([Formula: see text]) in SCD. Apo-A levels were inversely correlated with polyubiquitin levels ([Formula: see text], [Formula: see text]). 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.
journal_name
Pulm Circjournal_title
Pulmonary circulationauthors
Anjum F,Lazar J,Soh J,Albitar M,Gowda S,Hussain MM,Wadgaonkar Rdoi
10.1086/674763subject
Has Abstractpub_date
2013-12-01 00:00:00pages
851-5issue
4eissn
2045-8932issn
2045-8940pii
PC2013021journal_volume
3pub_type
杂志文章abstract::Pulmonary hypertension (PH) comprises a group of pathophysiological syndromes characterized by elevated pulmonary artery pressure and pulmonary vascular resistance, which lead to right ventricular overload, and even right heart failure. PH has a poor prognosis and severely leads to a decline in quality of life. Histor...
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abstract::[This corrects the article DOI: 10.1086/688060.]. ...
journal_title:Pulmonary circulation
pub_type: 已发布勘误
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