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先天性心脏畸形患儿心房中室肌球蛋白亚基的表达

Expression of ventricular myosin subunits in the atria of children with congenital heart malformations.

作者信息

Shi Q W, Danilczyk U, Wang J X, See Y P, Williams W G, Trusler G A, Beaulieu R, Rose V, Jackowski G

机构信息

Max Bell Research Center, Toronto General Hospital, Department of Clinical Biochemistry, University of Toronto, Ontario, Canada.

出版信息

Circ Res. 1991 Dec;69(6):1601-7. doi: 10.1161/01.res.69.6.1601.

DOI:10.1161/01.res.69.6.1601
PMID:1954679
Abstract

The presence of ventricular myosin light chains in the atria of children with congenital heart disease was demonstrated by two-dimensional polyacrylamide gel electrophoresis, peptide mapping, and Western blot analysis. Ventricular myosin light chains were present in 27% of biopsies from 91 children with different forms of congenital heart disease. Perimembranous ventricular septal defects and tetralogy of Fallot were associated with the presence of ventricular myosin light chains in 50% of patients. The presence of ventricular myosin light chains in these atria did not correlate with pressure or volume overload. Analysis of myosin heavy chain isotype in the same biopsies by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, peptide mapping, and Western blot analysis indicated that there was no detectable expression of ventricular myosin heavy chain (beta-subunit), suggesting that the genes for the myosin heavy chains and light chains are not expressed coordinately.

摘要

通过二维聚丙烯酰胺凝胶电泳、肽图谱分析和蛋白质免疫印迹分析,证实了先天性心脏病患儿心房中存在心室肌球蛋白轻链。在91例患有不同形式先天性心脏病的儿童活检组织中,27%检测到心室肌球蛋白轻链。膜周部室间隔缺损和法洛四联症患者中,50%的心房存在心室肌球蛋白轻链。这些心房中存在心室肌球蛋白轻链与压力或容量负荷无关。通过十二烷基硫酸钠-聚丙烯酰胺凝胶电泳、肽图谱分析和蛋白质免疫印迹分析对相同活检组织中的肌球蛋白重链同型进行分析,结果表明未检测到心室肌球蛋白重链(β亚基)的表达,这表明肌球蛋白重链和轻链的基因并非协同表达。

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Heavy and light roles: myosin in the morphogenesis of the heart.重链和轻链:肌球蛋白在心脏形态发生中的作用。
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The myocardial profile of the cytosolic isozymes of creatine kinase is apparently not related to cyanosis in congenital heart disease.
肌酸激酶胞浆同工酶的心肌谱与先天性心脏病中的发绀显然无关。
Mol Med. 1999 Feb;5(2):110-6.
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Coexpression of alpha and beta myosin heavy-chain isoforms in atria of neonates and infants with congenital heart disease.
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