Document Detail

Altered membrane proteins and permeability correlate with cardiac dysfunction in cardiomyopathic hamsters.
MedLine Citation:
PMID:  10749734     Owner:  NLM     Status:  MEDLINE    
A mutation in the delta-sarcoglycan (SG) gene with absence of delta-SG protein in the heart has been identified in the BIO14.6 cardiomyopathic (CM) hamster, but how the defective gene leads to myocardial degeneration and dysfunction is unknown. We correlated left ventricular (LV) function with increased sarcolemmal membrane permeability and investigated the LV distribution of the dystrophin-dystroglycan complex in BIO14.6 CM hamsters. On echocardiography at 5 wk of age, the CM hamsters showed a mildly enlarged diastolic dimension (LVDD) with decreased LV percent fractional shortening (%FS), and at 9 wk further enlargement of LVDD with reduction of %FS was observed. The percent area of myocardium exhibiting increased membrane permeability or membrane rupture, assessed by Evans blue dye (EBD) staining and wheat germ agglutinin, was greater at 9 than at 5 wk. In areas not stained by EBD, immunostaining of dystrophin was detected in CM hamsters at sarcolemma and T tubules, as expected, but it was also abnormally expressed at the intercalated discs; in addition, the expression of beta-dystroglycan was significantly reduced compared with control hearts. As previously described, alpha-SG was completely deficient in CM hearts compared with control hearts. In myocardial areas showing increased sarcolemmal permeability, neither dystrophin nor beta-dystroglycan could be identified by immunolabeling. Thus, together with the known loss of delta-SG and other SGs, abnormal distribution of dystrophin and reduction of beta-dystroglycan are associated with increased sarcolemmal permeability followed by cell rupture, which correlates with early progressive cardiac dysfunction in the BIO14.6 CM hamster.
Y Ikeda; M Martone; Y Gu; M Hoshijima; A Thor; S S Oh; K L Peterson; J Ross
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  American journal of physiology. Heart and circulatory physiology     Volume:  278     ISSN:  0363-6135     ISO Abbreviation:  Am. J. Physiol. Heart Circ. Physiol.     Publication Date:  2000 Apr 
Date Detail:
Created Date:  2000-04-27     Completed Date:  2000-04-27     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  100901228     Medline TA:  Am J Physiol Heart Circ Physiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  H1362-70     Citation Subset:  IM    
Division of Cardiology, Department of Medicine, University of California, San Diego, La Jolla, California 92093-0613, USA.
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MeSH Terms
Cardiomyopathies / metabolism*,  physiopathology*,  ultrasonography
Cell Membrane Permeability / physiology
Coloring Agents / pharmacokinetics
Cytoskeletal Proteins / analysis,  metabolism*
Disease Models, Animal
Dystrophin / analysis,  metabolism*
Evans Blue / pharmacokinetics
Heart Failure / metabolism,  physiopathology,  ultrasonography
Membrane Glycoproteins / analysis,  metabolism*
Microscopy, Immunoelectron
Myocardium / chemistry,  metabolism,  ultrastructure
Grant Support
Reg. No./Substance:
0/Coloring Agents; 0/Cytoskeletal Proteins; 0/Dystrophin; 0/Membrane Glycoproteins; 0/Sarcoglycans; 146888-27-9/Dystroglycans; 314-13-6/Evans Blue

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