|
| Status |
Public on Jun 02, 2021 |
| Title |
Long non-coding RNA lnc-TSSK2-8 activates canonical Wnt/β-catenin signaling through small shock proteins HSPA6 and CRYAB |
| Organism |
Homo sapiens |
| Experiment type |
Expression profiling by high throughput sequencing
|
| Summary |
Congenital heart defect (CHD) is the most common birth defect worldwide. Copy number variations (CNVs) have been revealed as an important source of pathogenic factor of CHD. 22q11.2 deletion syndrome is the most common microdeletion disorder which has been frequently associated with conotruncal malformations. By now, the dosage sensitive geneTBX1 has been adopted as the major pathogenic gene responsible for 22q11.2 deletion-related heart defects. Here we report the lncRNA lnc-TSSK2-8, which is encompassed in the 22q11.2 region, could activate the canonical Wnt/𝛃-catenin signaling by protecting 𝛃-catenin from ubiquitination and degradation. Such effects were mediated by two short heat shock proteins HSPA6 and CRYAB, whose expression were regulated by lnc-TSSK2-8 through the ceRNA mechanism. In clinical practice, pathogenesis of CNV were always attributed to haploinsufficiency of protein coding genes. Here we report the 22q11.2 lncRNA lnc-TSSK2-8 significantly activate canonical Wnt/ 𝛃-catenin signaling, which has major roles in cardiac out flow tract development and should act upstream of TBX1. Our results suggested that lncRNAs should contribute to the etiology of CNV related CHD.
|
| |
|
| Overall design |
TSSK2-8 overexpression
|
| |
|
| Contributor(s) |
Wang B, Fa J |
| Citation(s) |
34041241 |
| Submission date |
Feb 01, 2021 |
| Last update date |
Jun 02, 2021 |
| Contact name |
Bo Wang |
| E-mail(s) |
booew@163.com
|
| Organization name |
Shanghai Children's Medical Center
|
| Street address |
1678 Dongfang Road
|
| City |
Shanghai |
| ZIP/Postal code |
200127 |
| Country |
China |
| |
|
| Platforms (1) |
|
| Samples (6)
|
|
| Relations |
| BioProject |
PRJNA698585 |
| SRA |
SRP304142 |
Less...
More...