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. 2013 Nov 1;27(21):2320–2331. doi: 10.1101/gad.228957.113

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© 2013 Mansour et al.; Published by Cold Spring Harbor Laboratory Press

This article is distributed exclusively by Cold Spring Harbor Laboratory Press for the first six months after the full-issue publication date (see http://genesdev.cshlp.org/site/misc/terms.xhtml). After six months, it is available under a Creative Commons License (Attribution-NonCommercial 3.0 Unported), as described at http://creativecommons.org/licenses/by-nc/3.0/.

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Figure 6. — Model of FGFR3 signaling levels controlling differentiation of cochlear support cells. Normal support cell differentiation of two pillar cells and three Deiters' cells is depicted in the second column (boxed). The column at the far left shows cochlear differentiation when FGFR3 signaling is reduced; i.e., when Fgfr3 is globally absent or Fgf8 is conditionally absent in the Foxg1 domain, which includes the entire cochlear duct. Both conditions lead to incomplete or abnormal differentiation of pillar cells. The third column shows cochlear differentiation when FGFR3 signaling is increased by global deletion of Spry2. These cochleae have a cell fate transformation of one Deiters' cell to one pillar cell. The column at the far right shows cochlear differentiation when FGFR3 signaling is increased in the Muenke syndrome model (Fgfr3^P244R/+). These cochleae have a cell fate transformation of two Deiters' cells to two pillar cells. Rescue of the Spry2^−/− phenotype by Fgf8 heterozygosity and of the Fgfr3^P244R/+ phenotype by Fgf10 heterozygosity is depicted in the same column as the wild type (boxed). The genetic rescue results together with the L6 transfection assay data suggest that normal pillar cell differentiation is controlled by activation of FGFR3c by FGF8, whereas the Muenke syndrome model phenotypes arise by inappropriate activation of FGFR3b P244R and/or FGFR3c P244R by FGF10. Inner hair cells are colored in purple, outer hair cells are in blue, Fgf10-expressing cells are in gray, and support cells are in a shade of green depending on the level of FGFR3 signaling (light: low; dark: high). (DC) Deiters' cell; (IPC) inner pillar cell; (OPC) outer pillar cell.