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Fig. 9. Comparison of the C3Ggt/gt mutant phenotype with the
Reeler phenotype and the nervous-system-specific β1 integrin
conditional mutant phenotype. (A) At E14.5, the developing
wild-type mouse cortex consists of ventricular (VZ), subventricular (SVZ) and
intermediate (IZ) zones, subplate (SP), the nascent cortical plate (CP),
marginal zone (MZ) and has a continuous basement membrane (BM) at its pial
surface. Cajal-Retzius (CR, green) cells expressing reelin are located in the
marginal zone. Radial glial processes (RGP) extend from the ventricular zone
to the basement membrane at the pial surface, where they are anchored with
endfeet to the extracellular matrix protein lattice. Migrating bipolar neurons
(biN) are guided by radial glial processes and may transiently become
multipolar (muN). (B) β1 integrin deficiency in the brain leads to
a disruption of the basement membrane, loss of parallel radial glial process
orientation and loss of endfeet anchoring, as well as an undulating cortical
plate (U-CP) with invasion of the marginal zone by cortical plate cells
(Graus-Porta et al., 2001).
(C) Loss of reelin leads to a lack of reelin expression in the
Cajal-Retzius cells and to a failure of cortical plate cells to split the
preplate (PP) into marginal zone and subplate, resulting in an unsplit
preplate (US-PP) and the stacking of cortical plate cells (CPC) below the
unsplit preplate (Sheppard and Pearlman,
1997). In addition, lack of reelin leads to oblique orientation of
the radial glial processes. (D) The C3Ggt/gt mutant
phenotype combines all elements of the Reeler phenotype and the β1
integrin mutant phenotype: lack of preplate splitting, the stacking of
cortical plate cells below the unsplit preplate, disruption of the basement
membrane, invasion of the marginal zone and pericerebral space by cortical
plate cells, loss of radial glial process orientation and loss of endfeet
anchoring. In addition, lack of C3G leads to an accumulation of migrating
neurons in the multipolar stage.