Plexiform neurofibromas express the transcription factor Gli1.
BACKGROUND
Plexiform neurofibromas occur commonly in individuals with neurofibromatosis type 1 (NF1) and consist of neurofibromatous change in multiple nerve fascicles. Previously, we had observed that both plexiform neurofibromas and normal cutaneous nerves expressed Hedgehogs (Hhs), which are intercellular signaling molecules and regulate growth and patterning during embryonic development, and their receptors. In the present study, we examined the expression of Gli1, a transcription factor which mediates Hh signaling to investigate the activation of Hh signaling in plexiform neurofibromas and normal cutaneous nerves.


METHODS
An antihuman Gli1 antibody was used with a standard immunoperoxidase technique to determine Gli1 expression in 5 specimens of plexiform neurofibromas and 5 specimens of normal cutaneous nerves.


RESULTS
Our results showed Gli1 expression in S-100-positive tumor cells within the involved nerve fascicles in plexiform neurofibromas but not in control normal skins.


CONCLUSIONS
Our findings indicate that the Hh signaling pathway is activated in plexiform neurofibromas.
Endo
Hideharu
H
Department of Dermatology, Chiba Aoba Municipal Hospital, Chiba University, Aoba-cho,Chuo-ku, Japan. chw92121@syd.odn.ne.jp
Oikawa
Ayako
A
Utani
Atsushi
A
Shinkai
Hiroshi
H
D014408:Biomarkers, Tumor / Q000032:analysis*; D001707:Biopsy, Needle; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D018318:Neurofibroma, Plexiform / Q000235:genetics / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology*; D015513:Oncogene Proteins / Q000235:genetics / Q000378:metabolism*; D012016:Reference Values; D012680:Sensitivity and Specificity; D015398:Signal Transduction; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*; D046509:Tissue Culture Techniques; D015534:Trans-Activators; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism*; D000071676:Zinc Finger Protein GLI1
D014408:Biomarkers, Tumor; D015513:Oncogene Proteins; D015534:Trans-Activators; D014157:Transcription Factors; D000071676:Zinc Finger Protein GLI1