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Autoantibodies from mothers of children with congenital heart block downregulate cardiac L-type Ca channels.

Abstract：

: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.

journal_name

J Mol Cell Cardiol

journal_title

Journal of molecular and cellular cardiology

authors

Qu Y,Xiao GQ,Chen L,Boutjdir M

doi

10.1006/jmcc.2001.1379

keywords:

subject

Has Abstract

pub_date

2001-06-01 00:00:00

pages

1153-63

issue

6

eissn

0022-2828

issn

1095-8584

pii

S0022282801913793

journal_volume

33

pub_type

杂志文章

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