Fetal antigen 1, a member of the epidermal growth factor superfamily, in neurofibromas and serum from patients with neurofibromatosis type 1

Charlotte Harken Jensen, H D Schroder, B Teisner, I Laursen, F Brandrup, H B Rasmussen

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

Resumé

Fetal antigen 1 (FA1) is a 26-32 kDa glycoprotein containing six epidermal growth factor-like repeats closely related to the delta/notch/serrate proteins in Drosophila. FA1 has been shown to be involved in cell differentiation in a juxtacrine/paracrine manner. As neurofibromatosis type 1 (NF-1), also called von Recklinghausen disease, involves aberrant growth of tissues derived from the neural crest, the expression of FA1 was examined in neurofibroma skin biopsies and serum from patients with NF-1. FA1 was found in the spindle cells of all (n = 10) skin tumour specimens from adult NF-1 patients, whereas normal dermis was FA1 negative. In adults, the serum FA1 levels were significantly higher in NF-1 patients (n = 13) than in normal healthy controls (n = 177) (P = 0.037). In the group of children with NF-1 (n = 9), significantly higher serum FA1 levels were observed in those known to have complications with cerebral or spinal involvement (n = 4) (P = 0.014). The presence of FA1 in neurofibroma specimens and the elevated serum levels in patients with NF-1 suggests that FA1 may be involved in the pathogenesis of NF-1, perhaps acting as a growth promoting factor.
Udgivelsesdato: 1999-Jun
OriginalsprogEngelsk
TidsskriftBritish Journal of Dermatology
Vol/bind140
Udgave nummer6
Sider (fra-til)1054-9
Antal sider5
ISSN0007-0963
StatusUdgivet - 1999

Citer dette

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title = "Fetal antigen 1, a member of the epidermal growth factor superfamily, in neurofibromas and serum from patients with neurofibromatosis type 1",
abstract = "Fetal antigen 1 (FA1) is a 26-32 kDa glycoprotein containing six epidermal growth factor-like repeats closely related to the delta/notch/serrate proteins in Drosophila. FA1 has been shown to be involved in cell differentiation in a juxtacrine/paracrine manner. As neurofibromatosis type 1 (NF-1), also called von Recklinghausen disease, involves aberrant growth of tissues derived from the neural crest, the expression of FA1 was examined in neurofibroma skin biopsies and serum from patients with NF-1. FA1 was found in the spindle cells of all (n = 10) skin tumour specimens from adult NF-1 patients, whereas normal dermis was FA1 negative. In adults, the serum FA1 levels were significantly higher in NF-1 patients (n = 13) than in normal healthy controls (n = 177) (P = 0.037). In the group of children with NF-1 (n = 9), significantly higher serum FA1 levels were observed in those known to have complications with cerebral or spinal involvement (n = 4) (P = 0.014). The presence of FA1 in neurofibroma specimens and the elevated serum levels in patients with NF-1 suggests that FA1 may be involved in the pathogenesis of NF-1, perhaps acting as a growth promoting factor. Udgivelsesdato: 1999-Jun",
keywords = "Adolescent, Adult, Aged, Case-Control Studies, Child, Child, Preschool, Enzyme-Linked Immunosorbent Assay, Glycoproteins, Humans, Immunohistochemistry, Middle Aged, Neurofibroma, Neurofibromatosis 1, Skin, Skin Neoplasms, Statistics, Nonparametric",
author = "Jensen, {Charlotte Harken} and Schroder, {H D} and B Teisner and I Laursen and F Brandrup and Rasmussen, {H B}",
year = "1999",
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pages = "1054--9",
journal = "British Journal of Dermatology",
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Fetal antigen 1, a member of the epidermal growth factor superfamily, in neurofibromas and serum from patients with neurofibromatosis type 1. / Jensen, Charlotte Harken; Schroder, H D; Teisner, B; Laursen, I; Brandrup, F; Rasmussen, H B.

I: British Journal of Dermatology, Bind 140, Nr. 6, 1999, s. 1054-9.

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

TY - JOUR

T1 - Fetal antigen 1, a member of the epidermal growth factor superfamily, in neurofibromas and serum from patients with neurofibromatosis type 1

AU - Jensen, Charlotte Harken

AU - Schroder, H D

AU - Teisner, B

AU - Laursen, I

AU - Brandrup, F

AU - Rasmussen, H B

PY - 1999

Y1 - 1999

N2 - Fetal antigen 1 (FA1) is a 26-32 kDa glycoprotein containing six epidermal growth factor-like repeats closely related to the delta/notch/serrate proteins in Drosophila. FA1 has been shown to be involved in cell differentiation in a juxtacrine/paracrine manner. As neurofibromatosis type 1 (NF-1), also called von Recklinghausen disease, involves aberrant growth of tissues derived from the neural crest, the expression of FA1 was examined in neurofibroma skin biopsies and serum from patients with NF-1. FA1 was found in the spindle cells of all (n = 10) skin tumour specimens from adult NF-1 patients, whereas normal dermis was FA1 negative. In adults, the serum FA1 levels were significantly higher in NF-1 patients (n = 13) than in normal healthy controls (n = 177) (P = 0.037). In the group of children with NF-1 (n = 9), significantly higher serum FA1 levels were observed in those known to have complications with cerebral or spinal involvement (n = 4) (P = 0.014). The presence of FA1 in neurofibroma specimens and the elevated serum levels in patients with NF-1 suggests that FA1 may be involved in the pathogenesis of NF-1, perhaps acting as a growth promoting factor. Udgivelsesdato: 1999-Jun

AB - Fetal antigen 1 (FA1) is a 26-32 kDa glycoprotein containing six epidermal growth factor-like repeats closely related to the delta/notch/serrate proteins in Drosophila. FA1 has been shown to be involved in cell differentiation in a juxtacrine/paracrine manner. As neurofibromatosis type 1 (NF-1), also called von Recklinghausen disease, involves aberrant growth of tissues derived from the neural crest, the expression of FA1 was examined in neurofibroma skin biopsies and serum from patients with NF-1. FA1 was found in the spindle cells of all (n = 10) skin tumour specimens from adult NF-1 patients, whereas normal dermis was FA1 negative. In adults, the serum FA1 levels were significantly higher in NF-1 patients (n = 13) than in normal healthy controls (n = 177) (P = 0.037). In the group of children with NF-1 (n = 9), significantly higher serum FA1 levels were observed in those known to have complications with cerebral or spinal involvement (n = 4) (P = 0.014). The presence of FA1 in neurofibroma specimens and the elevated serum levels in patients with NF-1 suggests that FA1 may be involved in the pathogenesis of NF-1, perhaps acting as a growth promoting factor. Udgivelsesdato: 1999-Jun

KW - Adolescent

KW - Adult

KW - Aged

KW - Case-Control Studies

KW - Child

KW - Child, Preschool

KW - Enzyme-Linked Immunosorbent Assay

KW - Glycoproteins

KW - Humans

KW - Immunohistochemistry

KW - Middle Aged

KW - Neurofibroma

KW - Neurofibromatosis 1

KW - Skin

KW - Skin Neoplasms

KW - Statistics, Nonparametric

M3 - Journal article

C2 - 10354070

VL - 140

SP - 1054

EP - 1059

JO - British Journal of Dermatology

JF - British Journal of Dermatology

SN - 0007-0963

IS - 6

ER -