| First Author | Suzuki O | Year | 2011 |
| Journal | Proc Jpn Acad Ser B Phys Biol Sci | Volume | 87 |
| Issue | 8 | Pages | 550-62 |
| PubMed ID | 21986317 | Mgi Jnum | J:318028 |
| Mgi Id | MGI:6858153 | Doi | 10.2183/pjab.87.550 |
| Citation | Suzuki O, et al. (2011) Adult onset cardiac dilatation in a transgenic mouse line with Galbeta1,3GalNAc alpha2,3-sialyltransferase II (ST3Gal-II) transgenes: a new model for dilated cardiomyopathy. Proc Jpn Acad Ser B Phys Biol Sci 87(8):550-62 |
| abstractText | 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 Galbeta1,3GalNAc alpha2,3-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. |
