• Reproductive and Developmental Biology
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• 제35권4호
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• pp.457-461
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• 2011

JAZF1 (Juxtaposed with Another Zinc Finger gene 1) transcription factor are Zn-finger proteins that bind to the nuclear orphan receptor TAK/TR4 (Nakajima et al., 2004). The nuclear orphan receptor TAK1/TR4 functions as a positive as well as a negative regulator of transcription. It was recently reported that congenital cardiovascular malformations are significantly more frequent in Neurofibromatosis 1 (NF1) patients with microdeletion syndrome than in those with classical NF1. JAZF1 was expressed in adult heart of patients with microdeletion syndrome. JAZF1 is highly conserved among various species include zebrafish. We hypothesized that the expression of zebrafish Jazf1 may lead to severe forms of congenital heart disease that allow the survival of newborns and adults. In this study, we created Jazf1 transgenic zebrafish which over-express zebrafish Jazf1 cDNA under control of the CMV promoter. Our results suggested that Jazf1 expression may play an important role in zebrafish cardiac development.

• Journal of Chest Surgery
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• 제13권4호
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• pp.448-454
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• 1980

The juxtaposition of atrial appendage may possibly be diagnosed by angiography recently, and it is widely regarded as an ominous sign of severe cyanotic congenital heart disease. However, it is apparently rare congenital cardiac anomaly in which the atrial appendages lie side by side, both to the left or right of the great arteries, known as left or right juxtaposition of the atrial appendages. Juxtaposition of the atrial appendages has no functional significance, since it does not, itself, cause any hemodynamic disturbance. But it`s presence always indicates the coexistence of other major cardiac anomalies. In review of literatures TGA and VSD are invariable present, and ASD is common. Other anomalies, such as, tricuspid atresia, pulmonary outflow that, obstruction bicuspid pulmonic valve, persistent SVC etc. are relatively high incidence. In this report, we present one case of 6 year old female child having left juxtaposition of atrial appendage combined with TGA [D-looping, D-transposition], TAPVD, large ASD, small VSD, and vertical vein.

• Molecules and Cells
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• 제37권5호
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• pp.406-411
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• 2014

The initial step of atrioventricular (AV) valve development involves the deposition of extracellular matrix (ECM) components of the endocardial cushion and the endocardialmesenchymal transition. While the appropriately regulated expression of the major ECM components, Versican and Hyaluronan, that form the endocardial cushion is important for heart valve development, the underlying mechanism that regulates ECM gene expression remains unclear. We found that zebrafish crip2 expression is restricted to a subset of cells in the AV canal (AVC) endocardium at 55 hours post-fertilization (hpf). Knockdown of crip2 induced a heart-looping defect in zebrafish embryos, although the development of cardiac chambers appeared to be normal. In the AVC of Crip2-deficient embryos, the expression of both versican a and hyaluronan synthase 2 (has2) was highly upregulated, but the expression of bone morphogenetic protein 4 (bmp4) and T-box 2b (tbx2b) in the myocardium and of notch1b in the endocardium in the AVC did not change. Taken together, these results indicate that crip2 plays an important role in AV valve development by downregulating the expression of ECM components in the endocardial cushion.