{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T10:30:51Z","timestamp":1791196251849,"version":"4.1.0"},"reference-count":45,"publisher":"Springer Science and Business Media LLC","issue":"1","content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Transl Med"],"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:sec>\n            <jats:title>Background<\/jats:title>\n            <jats:p>Hypoxia and hypertrophy are the most frequent pathophysiological consequence of congenital heart disease (CHD) which can induce the alteration of Ca<jats:sup>2+<\/jats:sup>-regulatory proteins and inhibit cardiac contractility. Few studies have been performed to examine Ca<jats:sup>2+<\/jats:sup>-regulatory proteins in human cardiomyocytes from the hypertrophic right ventricle with or without hypoxia.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods<\/jats:title>\n            <jats:p>Right ventricle tissues were collected from children with tetralogy of Fallot [n\u2009=\u200925, hypoxia and hypertrophy group (HH group)], pulmonary stenosis [n\u2009=\u200925, hypertrophy group (H group)], or small isolated ventricular septal defect [n\u2009=\u200925, control group (C group)] during open-heart surgery. Paraffin sections of tissues were stained with 3,3\u2032-dioctadecyloxacarbocyanine perchlorate to measure cardiomyocyte size. Expression levels of Ca<jats:sup>2+<\/jats:sup>-regulatory proteins [sarcoplasmic reticulum Ca<jats:sup>2+<\/jats:sup>-ATPase (SERCA2a), ryanodine receptor (RyR2), sodiumcalcium exchanger (NCX), sarcolipin (SLN) and phospholamban (PLN)] were analysed by means of real-time PCR, western blot, or immunofluorescence. Additionally, phosphorylation level of RyR and PLN and activity of protein phosphatase (PP1) were evaluated using western blot.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>Mild cardiomyocyte hypertrophy of the right ventricle in H and HH groups was confirmed by comparing cardiomyocyte size. A significant reduction of SERCA2a in mRNA (<jats:italic>P<\/jats:italic>&lt;0.01) was observed in the HH group compared with the C group. The level of Ser<jats:sup>16<\/jats:sup>-phosphorylated PLN was down-regulated (<jats:italic>P<\/jats:italic>&lt;0.01) and PP1 was increased (<jats:italic>P<\/jats:italic>&lt;0.01) in the HH group compared to that in the C group.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>The decreased SERCA2a mRNA may be a biomarker of the pathological process in the early stage of cyanotic CHD with the hypertrophic right ventricle. A combination of hypoxia and hypertrophy can induce the adverse effect of PLN-Ser<jats:sup>16<\/jats:sup> dephosphorylation. Increased PP1 could result in the decreased PLN-Ser<jats:sup>16<\/jats:sup> and inhibition of PP1 is a potential therapeutic target for heart dysfunction in pediatrics.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1186\/1479-5876-10-67","type":"journal-article","created":{"date-parts":[[2012,4,2]],"date-time":"2012-04-02T10:14:41Z","timestamp":1333361681000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Ca2+-regulatory proteins in cardiomyocytes from the right ventricle in children with congenital heart disease"],"prefix":"10.1186","volume":"10","author":[{"given":"Yihe","family":"Wu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Feng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hao","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shoujun","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"De","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiangbin","family":"Pan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shengshou","family":"Hu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2012,4,2]]},"reference":[{"key":"1057_CR1","doi-asserted-by":"publisher","first-page":"2254","DOI":"10.1161\/CIRCULATIONAHA.110.947002","volume":"122","author":"SM Gilboa","year":"2010","unstructured":"Gilboa SM, Salemi JL, Nembhard WN, Fixler DE, Correa A: Mortality resulting from congenital heart disease among children and adults in the United States, 1999 to 2006. 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