Defective Desmosomal Adhesion Causes Arrhythmogenic Cardiomyopathy by Involving an Integrin-αVβ6/TGF-β Signaling Cascade | Zendy

Camilla Schinner | Zendy; Lifen Xu | Zendy; Henriette Franz | Zendy; Aude Zimmermann | Zendy; MarieTherès Wanuske | Zendy; Maitreyi Rathod | Zendy; Pauline Hanns | Zendy; Florian Geier | Zendy; Paweł Pelczar | Zendy; Yan Liang | Zendy; Vera Lorenz | Zendy; Chiara Stüdle | Zendy; I. Piotr Maly | Zendy; Silke Kauferstein | Zendy; Britt Maria Beckmann | Zendy; Farah Sheikh | Zendy; Gabriela M. Kuster | Zendy; Volker Spindler | Zendy

Open Access

Defective Desmosomal Adhesion Causes Arrhythmogenic Cardiomyopathy by Involving an Integrin-αVβ6/TGF-β Signaling Cascade

Author(s) -

Camilla Schinner,

Lifen Xu,

Henriette Franz,

Aude Zimmermann,

MarieTherès Wanuske,

Maitreyi Rathod,

Pauline Hanns,

Florian Geier,

Paweł Pelczar,

Yan Liang,

Vera Lorenz,

Chiara Stüdle,

I. Piotr Maly,

Silke Kauferstein,

Britt Maria Beckmann,

Farah Sheikh,

Gabriela M. Kuster,

Volker Spindler

Publication year - 2022

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.121.057329

Subject(s) - desmoplakin , medicine , integrin , microbiology and biotechnology , fibrosis , cell adhesion , desmosome , cell adhesion molecule , cancer research , pathology , immunology , biology , cell , genetics , receptor

Arrhythmogenic cardiomyopathy (ACM) is characterized by progressive loss of cardiomyocytes with fibrofatty tissue replacement, systolic dysfunction, and life-threatening arrhythmias. A substantial proportion of ACM is caused by mutations in genes of the desmosomal cell-cell adhesion complex, but the underlying mechanisms are not well understood. In the current study, we investigated the relevance of defective desmosomal adhesion for ACM development and progression.

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