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Review
. 2019 Jul;74(1):14-22.
doi: 10.1161/HYPERTENSIONAHA.119.12992. Epub 2019 May 28.

Pulmonary Hypertension and ATP-Sensitive Potassium Channels

Conor McClenaghan  1 , Kel Vin Woo  2 , Colin G Nichols  1
Affiliations
Review

Pulmonary Hypertension and ATP-Sensitive Potassium Channels

Conor McClenaghan et al. Hypertension. 2019 Jul.
No abstract available

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Conflict of interest statement

The authors have declared that no conflict of interest exists.

Figures

Fig. 1.
Fig. 1.. Molecular basis of KATP channel activity
(A) KATP channels are generated as octamers of 4 pore-forming Kir6.x (Kir6.1 or Kir6.2) and 4 regulatory SURx (SUR1 or SUR2) subunits. (B) Two pairs of genes located on human chromosome 12 (ABCC9, KCNJ8) and chromosome 11 (ABCC8, KCNJ11) encode SUR2 (C-terminally spliced to SUR2A or SUR2B) and Kir6.1, or SUR1 and Kir6.2 subunits, respectively.
Fig. 2.
Fig. 2.. Hypothesized mechanisms of KATP induced PH
(A) Schematic of cardiovascular system indicates normal pressures (grey) in systemic circulation and low pressures (blue) in pulmonary circulation resulting from normal pumping from the left (L) and right (R) heart, respectively. Loss-of-function of SUR1-dependent KATP (or other K) channels in pulmonary circulation may directly result in inappropriate pulmonary vasoconstriction and hypertension (red). (B) Gain-of-function of SUR2-dependent KATP (or other K) channels results primarily in inappropriate vasorelaxation and systemic hypotension (blue). Secondary compensatory mechanisms drive enlarged, hypercontractile hearts, raising pressures in the systemic circulation (to normal, grey) and in the pulmonary circulation (to hypertension, red).
See this image and copyright information in PMC

References

    1. George MG, Schieb LJ, Ayala C, Talwalkar A & Levant S Pulmonary hypertension surveillance: United States, 2001 to 2010. Chest 146, 476–495, doi:10.1378/chest.14-0527 (2014). - DOI - PMC - PubMed
    1. Simonneau G et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 43, 5S–12S, doi:10.1016/j.jacc.2004.02.037 (2004). - DOI - PubMed
    1. Simonneau G et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62, D34–41, doi:10.1016/j.jacc.2013.10.029 (2013). - DOI - PubMed
    1. Lane KB et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. Nat Genet 26, 81–84, doi:10.1038/79226 (2000). - DOI - PubMed
    1. Deng Z et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 67, 737–744, doi:10.1086/303059 (2000). - DOI - PMC - PubMed

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