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XB-ART-8754
J Mol Cell Cardiol 2001 Jun 01;336:1153-63. doi: 10.1006/jmcc.2001.1379.
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Autoantibodies from mothers of children with congenital heart block downregulate cardiac L-type Ca channels.

Qu Y , Xiao GQ , Chen L , Boutjdir M .


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Congenital heart block (CHB) affects offspring of mothers with autoantibodies (positive IgG) to intracellular SSA/Ro and SSB/La ribonucleoproteins and is associated with high morbidity and mortality. Here, we show that maternal anti-Ro/La antibodies immunoreact with human fetal cardiomyocyte sarcolemma, recognize human L-type Ca channel alpha(1C)-protein and functionally inhibit expressed current in oocytes injected with alpha(1C) cRNA and Purkinje L-type Ca current. Furthermore, cardiac myocytes from pups born to SSA/Ro-immunized mice exhibited reduced L-type Ca current density. All together, the data establish that L-type calcium channel is a target for maternal antibodies and may provide a functional basis for the electrocardiographic abnormalities seen in infants with CHB.

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???displayArticle.link??? J Mol Cell Cardiol
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