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Short Communication |
From Zentrum für Molekularbiologische Medizin,*
Universität zu Köln, Köln, and Abteilung
Neuropathologie,
Neurozentrum der
Albert-Ludwigs-Universität, Freiburg, Germany
Hemangioblastomas, the most frequent manifestation of the
hereditary von Hippel-Lindau disease (VHL), are highly
vascularized tumors of the central nervous system. In previous
studies, the endothelial-specific mitogen vascular endothelial
growth factor (VEGF) was shown to be up-regulated in the stromal
cells, the putative neoplastic cells in hemangioblastomas.
Therefore, it was suggested that secretion of VEGF by stromal
cells is the pathogenetic cause of the vascular lesions in
hemangioblastomas. The novel basic helix loop helix transcription
factor HRF/HIF-2
is a candidate regulator of VEGF expression during
development. We therefore investigated expression of HRF/HIF-2 in
hemangioblastomas and found the overexpression of VEGF mRNA in stromal
cells to be highly correlated with elevated expression levels of
HRF/HIF-2 mRNA. This finding is suggestive for a role of HRF in
VEGF-dependent vascular growth in hemangioblastomas and could provide a
link between transcriptional activation of the VEGF gene and loss of
function of the VHL gene product. (Am J Pathol 1998,
153:25–29)
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