Heart. 1996 Nov;76(5):412-7. doi: 10.1136/hrt.76.5.412.
Altered patterns of cardiac intercellular junction distribution in hypertrophic cardiomyopathy.
Heart (British Cardiac Society)
R Sepp, N J Severs, R G Gourdie
Affiliations
Affiliations
- Second Department of Internal Medicine, A Szent-Györgyi University of Medicine, Szeged, Hungary.
PMID: 8944586
PMCID: PMC484572 DOI: 10.1136/hrt.76.5.412
Free PMC Article
Abstract
OBJECTIVE: To examine the distribution pattern of intercellular junctions (the mechanically coupling desmosomes and the electrically coupling gap junctions) in hypertrophic cardiomyopathy (HCM) hearts showing myofibre disarray.
DESIGN: Samples from six necropsied hearts were studied, representing the interventricular septum and the free walls of the left and right ventricles. Immunohistochemical labelling of desmoplakin was used as a marker for desmosomes, and of connexin43 as a marker for gap junctions, in single and double stainings. The slides were examined by confocal laser scanning microscopy.
RESULTS: Marked disorganisation of intercalated discs was observed in areas featuring myofibre disarray. Besides overall derangement, localised abnormalities in desmosome organisation were evident, which included: (1) the formation of abnormally enlarged megadiscs; (2) the presence of intersecting disc structures; and (3) aberrant side to side desmosomal connections. Gap junctional abnormalities included: (1) random distribution of gap junctions over the surface of myocytes, rather than localisation to intercalated discs; (2) abundant side to side gap junction connections between adjacent myocytes; and (3) formation of abnormally shaped gap junctions. Circles of myocytes continuously interconnected by gap junctions were also observed. Regions of the diseased hearts lacking myofibre disarray, and control hearts of normal patients and patients with other cardiac diseases, did not show these alterations.
CONCLUSIONS: The disorganisation of the intercellular junctions associated with myofibre disarray in HCM may play an important role in the pathophysiological manifestations of the disease. The remodelling of gap junction distribution may underlie the formation of an arrhythmogenic substrate, thereby contributing to the generation and maintenance of cardiac arrhythmias associated with HCM.
Cited by
Pernot M, Jaspard-Vinassa B, Abelanet A, Rubin S, Forfar I, Jeanningros S, Cetran L, Yu MH, Balse E, Hatem S, Dufourcq P, Couffinhal T, Duplàa C.
Sci Rep. 2022 Jan 07;12(1):8. doi: 10.1038/s41598-021-03795-7.
PMID: 34996942
Pernot M, Jaspard-Vinassa B, Abelanet A, Rubin S, Forfar I, Jeanningros S, Cetran L, Yu MH, Balse E, Hatem S, Dufourcq P, Couffinhal T, Duplàa C.
Sci Rep. 2022 Jan 07;12(1):8. doi: 10.1038/s41598-021-03795-7.
PMID: 34996942
Pernot M, Jaspard-Vinassa B, Abelanet A, Rubin S, Forfar I, Jeanningros S, Cetran L, Yu MH, Balse E, Hatem S, Dufourcq P, Couffinhal T, Duplàa C.
Sci Rep. 2022 Jan 07;12(1):8. doi: 10.1038/s41598-021-03795-7.
PMID: 34996942
References
- J Microsc. 1993 Mar;169(Pt 3):299-328 - PubMed
- Circulation. 1993 May;87(5):1742-5 - PubMed
- J Cell Sci. 1993 Mar;104 ( Pt 3):741-50 - PubMed
- J Cell Sci. 1993 Aug;105 ( Pt 4):985-91 - PubMed
- Cell. 1994 Jun 3;77(5):701-12 - PubMed
- Circulation. 1994 Aug;90(2):713-25 - PubMed
- J Cardiovasc Electrophysiol. 1994 May;5(5):462-75 - PubMed
- J Morphol. 1994 Nov;222(2):203-13 - PubMed
- Br Heart J. 1994 Dec;72(6 Suppl):S10-2 - PubMed
- Clin Sci (Lond). 1995 Mar;88(3):257-62 - PubMed
- Nat Genet. 1995 Dec;11(4):434-7 - PubMed
- Nat Genet. 1995 Dec;11(4):438-40 - PubMed
- Circulation. 1974 Oct;50(4):809-20 - PubMed
- Am J Cardiol. 1977 Sep;40(3):355-64 - PubMed
- Circulation. 1979 Apr;59(4):689-706 - PubMed
- Circulation. 1979 Sep;60(3):685-96 - PubMed
- Cell. 1980 Mar;19(3):579-86 - PubMed
- Br Heart J. 1980 Oct;44(4):433-43 - PubMed
- Circulation. 1981 Apr;63(4):882-94 - PubMed
- Circulation. 1982 Jan;65(1):7-17 - PubMed
- Circ Res. 1985 Mar;56(3):436-51 - PubMed
- N Engl J Med. 1986 Sep 4;315(10):610-4 - PubMed
- N Engl J Med. 1987 Mar 26;316(13):780-9 - PubMed
- J Cell Biol. 1987 May;104(5):1389-402 - PubMed
- Circ Res. 1988 Jul;63(1):182-206 - PubMed
- N Engl J Med. 1989 Mar 23;320(12):749-55 - PubMed
- Circ Res. 1989 Jul;65(1):22-42 - PubMed
- Circulation. 1989 Nov;80(5):1489-92 - PubMed
- Int J Cardiol. 1990 Feb;26(2):137-73 - PubMed
- Cell. 1990 Sep 7;62(5):991-8 - PubMed
- Cell. 1990 Sep 7;62(5):999-1006 - PubMed
- Herz. 1991 Feb;16(1):13-21 - PubMed
- Am J Pathol. 1991 Oct;139(4):801-21 - PubMed
- Circulation. 1992 Jan;85(1):1-8 - PubMed
- Anat Embryol (Berl). 1992;185(4):363-78 - PubMed
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