Development 115, 289-303 (1992)
Printed in Great Britain © The Company of Biologists Limited 1992
289
Developmental expression of the a receptor for platelet-derived growth
factor, which is deleted in the embryonic lethal Patch mutation
AVI ORR-URTREGER
1
, MARK T. BEDFORD
1
, MYOUNG-SOOL DO
2
, LEA EISENBACH
2
and
PETER LONAI
1
*
Departments of ' Chemical Immunology and
2
Cell Biology, The Weumann Institute of Science, Rehovot 76100, Israel
•Author for correspondence
Summary
The a receptor of PDGF (Pdgfra) is expressed in
primitive endoderm and mesoderm derivatives through-
out embryogenesis. In the early primitive streak stage
the gene is transcribed in the visceral and parietal
endoderm. Later it is expressed in the presomitic
mesoderm, yolk sac and amnion. During somitogenesis
its transcription localizes to the heart and the somites.
Subsequently, it is transcribed in the dermatome, the
sclerotome, the developing limb and in various mes-
enchymal tissues of visceral organs. Its wild-type
expression pattern correlates well with the phenotype of
homozygous mutant Patch (Ph) embryos, where the
Pdgfra gene is deleted. The Ph phenotype is first
detectable at the primitive streak stage with convoluted
and hypertrophic visceral yolk sac, deformed neural
plate and disorganized or missing mesoderm. Most
Ph/Ph embryos die before the 11 day of gestation.
Those that survive till early organogenesis are very
small, have hypertrophic yolk sacs, small and undiffer-
entiated somites, convoluted neural tubes, large heart
and pericardium, rudimentary limb buds and branchial
arches. Our observations together suggest that the a
PDGF receptor may be required for the normal
development of visceral endoderm and mesoderm
derivatives.
Key words: PDGF receptor, embryogenesis, in situ
hybridization, Ph mutation, embryonic lethality
Introduction
PDGF (platelet-derived growth factor) is a major
mitogen for connective tissue and glial cells in vitro
(reviewed by Ross et al., 1986 and by Heldin and
Westermark, 1989). It stimulates chemotaxis, it is
possibly active in wound healing and it is thought to be
involved in the development of atherosclerotic plaques
(Rubin et al., 1988; Ferns et al., 1991). Abnormal
regulation of PDGF, due to mutation or overexpres-
sion, can contribute to malignant growth and both the B
and A chains of PDGF can cause malignant transform-
ation (Beckmann et al., 1988). A derivative of PDGF-
B, the \-sis oncogene, transforms cells that express the
PDGF receptor (Downward et al., 1984; Heldin and
Westermark, 1989). The role of PDGF in cell differen-
tiation was demonstrated in the glial cells of the optic
nerve, where PDGF is produced by type 1 astrocytes,
acts on bipotential glial progenitor cells, and is required
for the timing of their differentiation into oligodendro-
cytes (Noble et al., 1988; Raff et al., 1988).
PDGF is also expressed in embryonic carcinoma cells
(Gudas et al., 1983), m the extraembryonic membranes
and in the placenta (Goustin et al., 1985), suggesting
that it may have a role in embryogenesis. Numerous
other polypeptide growth factors, such as those belong-
ing to the FGF and the TGF/3 gene family, are active in
mesoderm induction, as detected by the Xenopus
animal cap model. PDGF however has no detectable
effect in this system (Smith, 1989; Whitman and
Melton, 1989), which may be one reason why we know
comparatively little about its role in early development.
Biochemical analysis revealed that PDGF and its
receptor (PDGF-R) each have two isoforms, desig-
nated A and B for the growth factor, a and /S for the
receptor. PDGF and PDGF-R isoforms function as
dimers, and both homo- and heterodimers are formed.
The a-receptor (Pdgfra) recognizes the A and B chains,
whereas the /S receptor (Pdgfrb) is specific for PDGF-B.
The PDGF AA homodimer is recognized exclusively by
the era-receptor homodimer (Seifert et al., 1989; Heldin
et al., 1988).
Recent evidence drew attention to the involvement
of Pdgfra in early development. Mercola et al. (1989)
showed that Pdgfra and PDGF-A are expressed prior to
Pdgfrb and PDGF-B in the 7.5- to 8.5-day-old mouse
embryo, suggesting that Pdgfra and PDGF-A may have
special roles at the primitive streak stage. RFLP