ArticleProceedings of the Japan Academy. Series B, Physical and biological sciences2011
Adult onset cardiac dilatation in a transgenic mouse line with Galβ1,3GalNAc α2,3-sialyltransferase II (ST3Gal-II) transgenes: a new model for dilated cardiomyopathy.
Article in Proceedings of the Japan Academy. Series B, Physical and biological sciences, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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9 citing papers in PubMed, 19 citations in OpenAlex.
- Development of Animal Models for Preclinical Safety Studies.Advances in experimental medicine and biology · 2025Review
- Alterations of the Sialylation Machinery in Brugada Syndrome.International journal of molecular sciences · 2022Article
- Analysis of the transgene insertion pattern in a transgenic mouse strain using long-read sequencing.Experimental animals · 2020Article
- Production of TRPV2-targeting functional antibody ameliorating dilated cardiomyopathy and muscular dystrophy in animal models.Laboratory investigation; a journal of technical methods and pathology · 2020Article
- TRPV2 channel as a possible drug target for the treatment of heart failure.Laboratory investigation; a journal of technical methods and pathology · 2020Review
- Remarkable Homeostasis of Protein Sialylation in Skeletal Muscles of Hibernating Daurian Ground Squirrels (Frontiers in physiology · 2020Article
- Blockade of TRPV2 is a Novel Therapy for Cardiomyopathy in Muscular Dystrophy.International journal of molecular sciences · 2019Review
- Novel inhibitor candidates of TRPV2 prevent damage of dystrophic myocytes and ameliorate against dilated cardiomyopathy in a hamster model.Oncotarget · 2018Article
- Calreticulin-STAT3 signaling pathway modulates mitochondrial function in a rat model of furazolidone-induced dilated cardiomyopathy.PloS one · 2013Article
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Authors and funding
11 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Sugar chain abnormalities in glycolipids and glycoproteins are associated with various diseases. Here, we report an adult onset cardiac dilatation in a transgenic mouse line with Galβ1,3GalNAc α2,3-sialyltransferase II (ST3Gal-II) transgenes. The transgenic hearts at the end-stage, at around 7 months old, were enlarged, with enlarged cavities and thin, low-tensile walls, typical of dilated cardiomyopathy. Although no apparent change was found in heart gangliosides, glycosylation of heart proteins was altered. Interestingly, sugar moieties not directly related to the ST3Gal-II catalytic reaction were also changed. Significant increases in calreticulin and calnexin were observed in hearts of the transgenic mice. These results suggest that expression of ST3Gal-II transgenes induces abnormal protein glycosylation, which disorganizes the endoplasmic/sarcoplasmic reticulum quality control system and elevates the calreticulin/calnexin level, resulting in suppression of cardiac function. The transgenic mice showed 100% incidence of adult onset cardiac dilatation, suggesting great potential as a new model for dilated cardiomyopathy.
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