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Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex
Journal article   Open access   Peer reviewed

Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex

Alessio Lancioni, Ida Luisa Rotundo, Yvonne Monique Kobayashi, Luca D'Orsi, Stefania Aurino, Gerardo Nigro, Giulio Piluso, Dario Acampora, Mafalda Cacciottolo, Kevin P Campbell, …
Human molecular genetics, Vol.20(23), pp.4644-4654
12/01/2011
DOI: 10.1093/hmg/ddr398
PMCID: PMC3209833
PMID: 21890494
url
https://doi.org/10.1093/hmg/ddr398View
Published (Version of record) Open Access

Abstract

Cardiomyopathy is a puzzling complication in addition to skeletal muscle pathology for patients with mutations in β-, γ- or δ-sarcoglycan (SG) genes. Patients with mutations in α-SG rarely have associated cardiomyopathy, or their cardiac pathology is very mild. We hypothesize that a fifth SG, ɛ-SG, may compensate for α-SG deficiency in the heart. To investigate the function of ɛ-SG in striated muscle, we generated an

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