A possible paracrine hedgehog signalling pathway in neurofibromas from patients with neurofibromatosis type 1

Endo, Hisayoshi; Matsumoto, Fumiaki; Kuroki, Toshio; Yoshimoto, Shinya; Ichinose, Masaharu; Shinkai, Hiroshi

doi:10.1046/j.1365-2133.2003.05178.x

Cited by 12 publications

(6 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…27 28 It is therefore of interest that a paracrine hedgehog signalling pathway has been implicated in the formation of neurofibromas in NF1. 29 The clinical and neurophysiological features in all our eight patients were of an indolent length dependent sensorimotor neuropathy with predominantly sensory signs. It was painless in seven out of eight patients.…”

Section: Discussionmentioning

confidence: 72%

Neurofibromatous neuropathy in neurofibromatosis 1 (NF1)

Ferner¹,

Hughes

Hall

et al. 2004

Journal of Medical Genetics

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 72%

Neurofibromatous neuropathy in neurofibromatosis 1 (NF1)

Ferner¹,

Hughes

Hall

et al. 2004

Journal of Medical Genetics

View full text Add to dashboard Cite

“…In our previous study [6], we did not detect positive immunoreactivity for Ptch, another Hh target gene [3]on neurofibromas. This apparent inconsistency with the present study was probably due to technical problems, since neither neurofibromas nor BCCs [5]were positive for Ptch by the methods described in the previous study (data not shown).…”

Section: Discussionmentioning

confidence: 99%

“…This suggests that Hhs within the nerve fascicles, which are probably secreted from the perineuria [6], stimulate tumor cells to activate the pathway. Therefore, the Hh signaling pathway may be required only for the intraneural proliferation of tumor cells, and there are likely other mechanisms for the extraneural extension of the tumor cells.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Plexiform Neurofibromas Express the Transcription Factor Gli1

2004

View full text Add to dashboard Cite

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.

show abstract

“…Expression of Hh members has also been shown in various tumors. Especially, recent studies have suggested that an Hh signaling pathway may be involved in tumorigenesis of NFs and MPNSTs in NF1 [14][15][16][17][18]. The transcription factor Gli1, which mediates Hh signaling, is indeed expressed in plexiform NF suggesting that Hh pathways are activated in these tumors.…”

Section: Introductionmentioning

confidence: 99%