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Plexiform neurofibromas express the transcription factor Gli1
Hideharu Endo1, Ayako Oikawa, Atsushi Utani
1Department of Dermatology, Chiba Aoba Municipal Hospital, Chiba University, Aoba-cho,Chuo-ku, Japan. chw92121@syd.odn.ne.jp
Dermatology (Basel, Switzerland)
|November 13, 2004
Summary
Hedgehog (Hh) signaling is activated in plexiform neurofibromas, common in neurofibromatosis type 1 (NF1). Gli1 expression in tumor cells indicates this pathway
Area of Science:
- Oncology
- Molecular Biology
- Genetics
Background:
- Plexiform neurofibromas are common in neurofibromatosis type 1 (NF1).
- Hedgehog (Hh) signaling molecules and receptors are expressed in neurofibromas and normal nerves.
- Gli1 mediates Hh signaling, regulating growth and development.
Purpose of the Study:
- To investigate the activation of the Hh signaling pathway in plexiform neurofibromas.
- To examine Gli1 expression as an indicator of Hh pathway activation.
Main Methods:
- Immunohistochemistry using an anti-human Gli1 antibody.
- Standard immunoperoxidase technique.
- Analysis of 5 plexiform neurofibroma and 5 normal cutaneous nerve specimens.
Main Results:
- Gli1 expression was detected in S-100-positive tumor cells within plexiform neurofibromas.
- Gli1 expression was absent in normal cutaneous nerves (control).
Conclusions:
- The Hedgehog (Hh) signaling pathway is activated in plexiform neurofibromas.
- Gli1 is a potential marker for Hh pathway activation in NF1-associated tumors.