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Expression of vitamin A in peripheral blood and differential expression of retinoic acid receptor α and HA117 in colon segments of children with Hirschsprung’s disease
LUO Yuan-yuan TENG Yin-ping LIU Hang LI Li JIN Xian-qing*
Acta Anatomica Sinica ›› 2015, Vol. 46 ›› Issue (4) : 558-563.
Expression of vitamin A in peripheral blood and differential expression of retinoic acid receptor α and HA117 in colon segments of children with Hirschsprung’s disease
Objective To describe the expression profiles of vitamin A in blood serum and retinoic acid receptor α（RARα）, HA117 in different portions of the colon of patients diagnosed with Hirschsprung’s disease (HD). Methods Peripheral blood and colon specimens were collected from 17 HD patients. The concentration of vitamin A was detected in blood serum, and the colon specimens were grouped into 3 segments: anastomosis, dilated segment and stenotic segment. Levels of RARα mRNA and HA117 RNA were evaluated by Realtime PCR. RARα protein expression were analyzed by immunohistochemistry and Western blotting. Results The concentration of vitamin A in peripheral blood is (0.73±0.05) μmol/L, which was below the normal level. The levels of RARα mRNA expression was significantly lower in the stenotic segment compared to proximal anastomosis (P＜0.05,n=17). The level of HA117 RNA expression was significantly higher in the stenotic segment compared to proximal anastomosis (P＜0.001,n=17). RARα were expressed in ganglion cells and nerve fibers between the circular and longitudinal muscles in anastomosis; positive staining was diminished in the stenotic segments. The levels of RARα protein expression was significantly lower in the dilated segment（P＜0.05,n=17）and stenotic segment（P＜0.001, n=17）compared to proximal anastomosis. Conclusion The vitamin A and RARα may contribute to the development of enteric nervous system (ENS); long non-coding RNA (LncRNA) HA117 may have an anti-differentiation function, and play a pivotal role in the progression of HD.
Hirschsprung’s disease / Vitamin A / Retinoic acid receptor α / / HA117 / / Real-time PCR / Immunohistochemistry / Western blotting / Child
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