Serum Caveolin-1 as a Novel Biomarker in Idiopathic Pulmonary Artery Hypertension

Wang, Kuo‐Yang; Lee, Mey-Fann; Ho, Hung-Chin; Liang, Kae‐Woei; Liu, Chia‐Chi; Tsai, Wan-Jane; Lin, Wei‐Wen

doi:10.1155/2015/173970

Cited by 15 publications

(16 citation statements)

References 32 publications

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“…[22][23][24] In addition, human studies support the role of CAV1 in PAH, with reduced caveolin-1 expression found to occur in the lungs of PAH patients regardless of mutation, and reduced systemic caveolin-1 levels. [25][26][27] A recent study found a CAV1 mutation in a child also with lipodystrophy, connecting these two metabolic disorders. 28 However, the manner by which CAV1 mutations promote PAH pathogenesis in humans remains incompletely elucidated.…”

Section: Additional Genes Believed To Cause Pah When Pathogenic Mutatmentioning

confidence: 98%

Genetics of Pulmonary Arterial Hypertension

Chew

Loyd

Austin

2017

Semin Respir Crit Care Med

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Tremendous progress has been made in understanding the genetics of pulmonary arterial hypertension (PAH) since its description in the 1950s as a primary disorder of the pulmonary vasculature. Heterozygous germline mutations in the gene coding bone morphogenetic receptor type 2 (BMPR2) are detectable in the majority of cases of heritable PAH, and in approximately 20% of cases of idiopathic pulmonary arterial hypertension (IPAH). However, recent advances in gene discovery methods have facilitated the discovery of additional genes with mutations among those with and without familial PAH. Heritable PAH is an autosomal dominant disease characterized by reduced penetrance, variable expressivity, and female predominance. Biallelic germline mutations in the gene EIF2AK4 are now associated with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Growing genetic knowledge enhances our capacity to pursue and provide genetic counseling, although the issue remains complex given that the majority of carriers of PAH-related mutations will never be diagnosed with the disease.

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Section: Additional Genes Believed To Cause Pah When Pathogenic Mutatmentioning

confidence: 98%

Genetics of Pulmonary Arterial Hypertension

Chew

Loyd

Austin

2017

Semin Respir Crit Care Med

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“…33 Genetic deletion of caveolin-1 abrogates endotheliumderived hyperpolarizing factor (EDHF)-induced hyperpolarization by altering calcium entry, thus highlighting the role of caveolin-1 in EDHF regulation. 34 Caveolin-1 inhibits a number of proliferative pathways. Downregulation of caveolin-1 leads to hyperactivation of mitogen-activated protein kinase (MAPK)/extracellular signal-regulated kinase (ERK) pathway.…”

Section: Caveolin-1mentioning

confidence: 99%

Pulmonary artery hypertension in childhood: The transforming growth factor-β superfamily-related genes

Yuan

2018

Pediatrics & Neonatology

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Pulmonary artery hypertension (PAH) is very rare in childhood, and it can be divided into heritable, idiopathic drug- and toxin-induced and other disease (connective tissue disease, human immunodeficiency virus infection, portal hypertension, congenital heart disease, or schistosomiasis)-associated types. PAH could not be interpreted solely by pathophysiological theories. The impact of the transforming growth factor-β superfamily-related genes on the development of PAH in children remains to be clarified. Pertinent literature on the transforming growth factor-β superfamily-related genes in relation to PAH in children published after the year 2000 was reviewed and analyzed. Bone morphogenetic protein receptor type II gene mutation promotes cell division or prevents cell death, resulting in an overgrowth of cells in small arteries throughout the lungs. About 20% of individuals with a bone morphogenetic protein receptor type II gene mutation develop symptomatic PAH. In heritable PAH, bone morphogenetic protein receptor type II mutations may be absent; while mutations of other genes, such as type I receptor activin receptor-like kinase 1 and the type III receptor endoglin (both associated with hereditary hemorrhagic telangiectasia), caveolin-1 and KCNK3, the gene encoding potassium channel subfamily K, member 3, can be detected, instead. Gene mutations, environmental changes and acquired adjustment, etc. may explain the development of PAH. The researches on PAH rat model and familial PAH members may facilitate the elucidations of the mechanisms and further provide theories for prophylaxis and treatment of PAH.

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“…However, its secretion in vascular endothelial cells is decreased in patients with PH, and a serum cutoff of 17.17 pg/mL is highly suggestive of idiopathic PH. 14 …”

Section: Use Of Biomarkers In Phmentioning

confidence: 99%

“…However, its secretion in vascular endothelial cells is decreased in patients with PH, and a serum cutoff of 17.17 pg/mL is highly sugges tive of idiopathic PH. 14 Elastin is a structural constituent of blood vessels, and its synthesis requires crosslinking of monomers by two amino acids, desmosine and isodesmosine. In patients with PH, elas tin turnover is elevated, and consequently, plasma and urine levels of desmosine and isodesmosine are high.…”

Section: Use Of Biomarkers In Phmentioning

confidence: 99%

Role of Biomarkers in the Diagnosis, Risk Assessment, and Management of Pulmonary Hypertension

Rameh

Kossaify

2016

Biomark�Insights

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Pulmonary hypertension is a severe and debilitating disease with no definite cure, and the domain of targeted therapies is a promising field for better management of this severe condition. The disease comprises pulmonary arterial remodeling, hypoxia, endothelial dysfunction, and inflammation, with subsequent organ damage including right heart and liver dysfunction. Biomarkers have a valuable role at different levels of the disease, from diagnosis to risk assessment and management, in order to decrease the burden of the disease in terms of both morbidity and mortality.

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Serum Caveolin-1 as a Novel Biomarker in Idiopathic Pulmonary Artery Hypertension

Cited by 15 publications

References 32 publications

Genetics of Pulmonary Arterial Hypertension

Genetics of Pulmonary Arterial Hypertension

Pulmonary artery hypertension in childhood: The transforming growth factor-β superfamily-related genes

Role of Biomarkers in the Diagnosis, Risk Assessment, and Management of Pulmonary Hypertension

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