Human Molecular Genetics, Vol 8, 229-236, Copyright © 1999 by Oxford University Press
M Hemberger, H Himmelbauer, HP Neumann, KH Plate, G Schwarzkopf and R Fundele
The von Hippel-Lindau (VHL) tumour suppressorgene product is believed to be
involved in the down-regulation of transcriptional elongation by preventing
the association of elongin B and C with the catalytic subunit elongin A.
Alterations in the human VHL gene lead to VHL disease which is associated
with various rare neoplasias, including haemangioblastoma of the central
nervous system, retinal angioma, clear cell renal carcinoma and
pheochromocytoma. Recently, a protein (VBP1) was isolated that was found to
bind to the VHL protein in vivo. We have used the murine Vbp1 homologous
cDNA to investigate the expression of the Vbp1 mRNA in the mouse by in situ
hybridization and northern blot analysis. In fetal stages between days 9
and 18 of gestation, Vbp1 was expressed mainly in the central nervous
system, retina and liver. In addition, at day 12, high expression was
observed in the labyrinthine region of the placenta. In later stage
placentas, Vbp1 expression was, however, considerably reduced. Northern
blot analysis of adult mouse tissues showed that Vbp1 was ubiquitously
expressed. In situ analysis on several adult tissues showed that in most
tissues, transcripts were evenly distributed. In brain, eye, kidney and
intestine, however, Vbp1 was expressed in specific cell types. Moreover,
expression of the human VBP1 gene was investigated in cerebellum and in
various tumours of VHL patients encompassinghaemangioblastomas, renal cell
carcinomas and pheochromocytomas. In all of these tissues, VBP1 was
ubiquitously expressed at low levels. However, no consistent differences in
VBP1 expression levels could be detected between tumours and normal tissue.
Mapping of the murine Vbp1 gene revealed conserved chromosomal localization
between mouse and human in a region homologous to human Xq28.
ARTICLES
Expression of the von Hippel-Lindau-binding protein-1 (Vbp1) in fetal and adult mouse tissues
Max-Planck-Institut fur Molekulare Genetik, Ihnestrasse 73, D-14195 Berlin-Dahlem, Germany, Fakultat fur Biologie III, Universitat Freiburg, Freiburg, Germany.
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