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PMID:17404841

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Citation

Thomas, SL and De Vries, GH (2007) Angiogenic expression profile of normal and neurofibromin-deficient human Schwann cells. Neurochem. Res. 32:1129-41

Abstract

Peripheral nerve sheath tumors from individuals with Neurofibromatosis Type 1 (NF1) are highly vascular and contain Schwann cells which are deficient in neurofibromin. This study examines the angiogenic expression profile of neurofibromin-deficient human Schwann cells relative to normal human Schwann cells, characterizing both pro-angiogenic and anti-angiogenic factors. Conditioned media from neurofibromin-deficient Schwann cell lines was pro-angiogenic as evidenced by its ability to stimulate endothelial cell proliferation and migration. Using gene array and protein array analysis, we found increased expression of pro-angiogenic factors and decreased expression of anti-angiogenic factors in neurofibromin-deficient Schwann cells relative to normal human Schwann cells. Neurofibromin-deficient Schwann cells also showed increased expression of several growth factor receptors and decreased expression of an integrin. We conclude that neurofibromin-deficient Schwann cells have dysregulated expression of pro-angiogenic factors, anti-angiogenic factors, growth factor receptors, and an integrin. These dysregulated molecules may contribute to the growth and progression of NF1 peripheral nerve sheath tumors.

Links

PubMed Online version:10.1007/s11064-007-9279-z

Keywords

Angiogenesis Inhibitors/genetics; Angiogenesis Inhibitors/metabolism; Angiogenic Proteins/genetics; Angiogenic Proteins/metabolism; Cell Movement/physiology; Cell Proliferation; Culture Media, Conditioned; Endothelial Cells/cytology; Endothelial Cells/physiology; Gene Expression Profiling; Humans; Molecular Sequence Data; Neovascularization, Pathologic; Neovascularization, Physiologic; Neurofibromatosis 1/genetics; Neurofibromatosis 1/metabolism; Neurofibromatosis 1/pathology; Neurofibromin 1/genetics; Neurofibromin 1/metabolism; Oligonucleotide Array Sequence Analysis; Protein Array Analysis; Schwann Cells/cytology; Schwann Cells/physiology

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:NF1

involved_in

GO:0045765: regulation of angiogenesis

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:NF1

involved_in

GO:0043535: regulation of blood vessel endothelial cell migration

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:NF1

involved_in

GO:0001937: negative regulation of endothelial cell proliferation

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete


See also

References

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