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Updated: Dec 18, 2025

Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome
Published on: October 3, 2018
Sonic hedgehog signaling pathway in Myelodysplastic Syndrome: Abnormal activation and jervine intervention
YuTing Qin1, Ming Jiang1, Nilupar Tuerxung1
1Hematologic Disease Center, The First Affiliated Hospital of Xinjiang Medical University, No. 137, Liyushan Road, Xinshi District, Urumqi, Xinjiang Province 830054, China.
Objective:
This study aims to investigate the roles of Sonic hedgehog (Shh) signaling pathway in the occurrence and progression of Myelodysplastic Syndrome (MDS) and further evaluate using jervine as therapeutic strategy for MDS by inhibiting Shh pathway.
Methods:
CD34+ cells from the bone marrow of 53 MDS patients were counted by flow cytometry and isolated by magnetic bead sorting. Shh, Smo, Ptch-1 and Gli-1 (involved in Shh pathway) in CD34+ cells were examined by RT-qPCR. Besides, the relationship between Shh pathway-related genes and the clinical features or prognosis of MDS were analyzed. Further, the effects of jervine on MUTZ-1 cells regarding their proliferation, apoptosis and cell cycle as well as Shh pathway-related gene and protein expression were analyzed.
Results:
Gene expression level of Shh, Gli-1 and Smo was significantly increased in MDS patients. Herein, Smo and Gli-1 were correlated with chromosome karyotype classification and IPSS. MDS patients with high expression of Smo or Gli-1 had a poor prognosis. Jervine inhibited gene and protein expression of Shh, Smo, Ptch-1 and Gli-1. Besides, jervine suppressed the proliferation and promoted the apoptosis of MUTZ-1 cells, as well as inhibited the transition of cells from G1 to S phase.
Conclusion:
Shh signaling pathway of MDS patients is abnormally activated and participated in the occurrence and progression of MDS. Jervine intervention is a potential therapeutic strategy for MDS.
Insights
The Sonic hedgehog (Shh) signaling pathway is activated in Myelodysplastic Syndrome (MDS). Inhibiting this pathway with jervine shows potential as a therapeutic strategy for MDS patients.
Area of Science:
- Hematology
- Molecular Biology
- Oncology
Background:
- Myelodysplastic Syndrome (MDS) is a group of clonal hematopoietic stem cell disorders.
- The Sonic hedgehog (Shh) signaling pathway plays a role in various cellular processes, including development and disease.
Purpose of the Study:
- To investigate the role of the Shh signaling pathway in MDS occurrence and progression.
- To evaluate jervine as a potential therapeutic agent for MDS by targeting the Shh pathway.
Main Methods:
- Analysis of Shh pathway components (Shh, Smo, Ptch-1, Gli-1) in bone marrow CD34+ cells from MDS patients using RT-qPCR.
- Correlation analysis of Shh pathway gene expression with clinical features and prognosis.
- Assessment of jervine's effects on MUTZ-1 cell proliferation, apoptosis, cell cycle, and Shh pathway gene/protein expression.
Main Results:
- Significantly increased expression of Shh, Gli-1, and Smo in MDS patients.
- Correlation of Smo and Gli-1 expression with chromosome karyotype and IPSS, indicating a link to poor prognosis.
- Jervine inhibited Shh pathway gene/protein expression, suppressed proliferation, promoted apoptosis, and arrested the cell cycle in MUTZ-1 cells.
Conclusions:
- Abnormal activation of the Shh signaling pathway is implicated in MDS pathogenesis.
- Jervine demonstrates potential as a therapeutic strategy for MDS by modulating the Shh pathway.
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