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Updated: Mar 9, 2026

Quantitative PCR-based Assay to Measure Sonic Hedgehog Signaling in Cellular Model of Ciliogenesis
Published on: January 31, 2025
Small GTPase Arl6 controls RH30 rhabdomyosarcoma cell growth through ciliogenesis and Hedgehog signaling
Xiaotong Liu1, Qiuhong Shen1, Tingting Yu1
1Department of Developmental Genetics, School of Basic Medical Sciences, Nanjing Medical University, Nanjing, 211166 Jiangsu People's Republic of China.
Background:
Rhabdomyosarcoma (RMS) originates from skeletal muscle precursors that fail to differentiate. Hedgehog (Hh) signaling and primary cilia contribute to the pathobiology of RMS.
Results:
Here we showed ADP ribosylation factor like GTPase 6 (ARL6) localizes at the base of primary cilium, controls ciliogenesis and Hh signaling. The transcription of Arl6 is dynamic during the differentiation of myoblasts, companying with the growth and elimination of primary cilia. Arl6 expression is significantly up regulated in cilia-dependent RMS cells and tissues. Knockdown of Arl6 inhibits proliferation and promotes apoptosis of RMS RH30 cells through defected ciliogenesis and reduced Hh activity.
Conclusions:
Taken together, the functions of Arl6 in ciliogenesis and Hh signaling suggest it as a potential RMS drug target.
Insights
ADP ribosylation factor like GTPase 6 (ARL6) is crucial for primary cilia formation and Hedgehog signaling in rhabdomyosarcoma. Targeting ARL6 may offer a new therapeutic strategy for this cancer.
Area of Science:
- Cell Biology
- Molecular Oncology
- Cancer Research
Background:
- Rhabdomyosarcoma (RMS) arises from undifferentiated skeletal muscle precursors.
- Hedgehog (Hh) signaling and primary cilia are implicated in RMS development.
Purpose of the Study:
- To investigate the role of ADP ribosylation factor like GTPase 6 (ARL6) in ciliogenesis and Hh signaling within the context of RMS.
Main Methods:
- Localization studies of ARL6 at primary cilia.
- Analysis of Arl6 transcription during myoblast differentiation.
- Assessment of ARL6 expression in RMS cells and tissues.
- Investigating the effects of Arl6 knockdown on RMS cell proliferation, apoptosis, ciliogenesis, and Hh activity.
Main Results:
- ARL6 localizes to the base of primary cilia and regulates ciliogenesis and Hh signaling.
- Arl6 transcription changes dynamically with myoblast differentiation and primary cilia dynamics.
- ARL6 expression is elevated in cilia-dependent RMS cells and tissues.
- ARL6 knockdown in RH30 RMS cells reduced proliferation and increased apoptosis via impaired ciliogenesis and Hh signaling.
Conclusions:
- ARL6 plays a significant role in ciliogenesis and Hh signaling.
- The involvement of ARL6 in these processes suggests its potential as a therapeutic target for rhabdomyosarcoma.
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