Development and degeneration of dorsal root ganglia in the absence of the HMG-domain transcription factor Sox10

Sonnenberg-Riethmacher, Eva; Miehe, Michaela; Stolt, C. Claus; Goerich, Derk E.; Wegner, Michael; Riethmacher, Dieter

doi:10.1016/s0925-4773(01)00547-0

Cited by 92 publications

(78 citation statements)

References 60 publications

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“…In the neural crest, sox10 has been shown to have a role in sensory neuron specification and survival (Britsch et al, 2001;Sonnenberg-Riethmacher et al, 2001;Carney et al, 2006). Hence, we asked whether sox10 mutants might show disrupted sensory neuron formation in the SAG.…”

Section: The Neurogenic Domain Is Expanded In Cls/sox10 Mutant Earsmentioning

confidence: 99%

A zebrafish model for Waardenburg syndrome type IV reveals diverse roles for Sox10 in the otic vesicle

Dutton

Abbas

Spencer

et al. 2009

Disease Models &Amp; Mechanisms

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SUMMARYIn humans, mutations in the SOX10 gene are a cause of the auditory-pigmentary disorder Waardenburg syndrome type IV (WS4) and related variants. SOX10 encodes an Sry-related HMG box protein essential for the development of the neural crest; deafness in WS4 and other Waardenburg syndromes is usually attributed to loss of neural-crest-derived melanocytes in the stria vascularis of the cochlea. However, SOX10 is strongly expressed in the developing otic vesicle and so direct roles for SOX10 in the otic epithelium might also be important. Here, we examine the otic phenotype of zebrafish sox10 mutants, a model for WS4. As a cochlea is not present in the fish ear, the severe otic phenotype in these mutants cannot be attributed to effects on this tissue. In zebrafish sox10 mutants, we see abnormalities in all otic placodal derivatives. Gene expression studies indicate deregulated expression of several otic genes, including fgf8, in sox10 mutants. Using a combination of mutant and morphant data, we show that the three sox genes belonging to group E (sox9a, sox9b and sox10) provide a link between otic induction pathways and subsequent otic patterning: they act redundantly to maintain sox10 expression throughout otic tissue and to restrict fgf8 expression to anterior macula regions. Single-cell labelling experiments indicate a small and transient neural crest contribution to the zebrafish ear during normal development, but this is unlikely to account for the strong defects seen in the sox10 mutant. We discuss the implication that the deafness in WS4 patients with SOX10 mutations might reflect a haploinsufficiency for SOX10 in the otic epithelium, resulting in patterning and functional abnormalities in the inner ear.

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Section: The Neurogenic Domain Is Expanded In Cls/sox10 Mutant Earsmentioning

confidence: 99%

A zebrafish model for Waardenburg syndrome type IV reveals diverse roles for Sox10 in the otic vesicle

Dutton

Abbas

Spencer

et al. 2009

Disease Models &Amp; Mechanisms

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show abstract

“…Thus, homozygous Sox10 mutants lack early markers of PNS glial progenitors (e.g. Erbb3 and Notch1), as well as glial differentiation markers (Britsch et al, 2001;Sonnenberg-Riethmacher et al, 2001). Complete lack of Sox10 function results in cell death before overt fate acquisition, and any surviving cells fail to subsequently differentiate as glia (Britsch et al, 2001;Kapur, 1999;Paratore et al, 2001).…”

Section: Introductionmentioning

confidence: 99%

“…Ngn genes are expressed in a subset of NCCs early during migration, but are rapidly downregulated in the nascent DRGs (Greenwood et al, 1999;Perez et al, 1999). DRG neuron specification was not examined in mouse Sox10 mutants (Britsch et al, 2001;Sonnenberg-Riethmacher et al, 2001). Hence, whether Sox10 mutants show an early reduction in nascent sensory neurons, in addition to later neuronal death due to absence of glial support remains untested.…”

Section: Introductionmentioning

confidence: 99%

A direct role for Sox10 in specification of neural crest-derived sensory neurons

et al. 2006

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sox10 is necessary for development of neural and pigment cell derivatives of the neural crest (NC). However, whereas a direct role for Sox10 activity has been established in pigment and glial lineages, this is more controversial in NC-derived sensory neurons of the dorsal root ganglia (DRGs). We proposed that sox10 functioned in specification of sensory neurons, whereas others suggested that sensory neuronal defects were merely secondary to absence of glia. Here we provide evidence that in zebrafish, early DRG sensory neuron survival is independent of differentiated glia. Critically, we demonstrate that Sox10 is expressed transiently in the sensory neuron lineage, and specifies sensory neuron precursors by regulating the proneural gene neurogenin1. Consistent with this, we have isolated a novel sox10 mutant that lacks glia and yet displays a neurogenic DRG phenotype. In conjunction with previous findings, these data establish the generality of our model of Sox10 function in NC fate specification.

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“…Sox10 expression starts in premigratory NC cells and continues in migrating NC cells and NC cell-derivatives (Mollaaghababa and Pavan, 2003); however, loss of Sox10 function does not affect NC formation and migration. Mice lacking Sox10 have NC cells that undergo apoptosis before reaching their maturation stage Southard-Smith et al, 1998;Britsch et al, 2001;Paratore et al, 2001;Sonnenberg-Riethmacher et al, 2001). Dominant megacolon mice (Dom), which have a spontaneous mutation in their Sox10 locus, have pigmentation defects and intestinal aganglionosis, which are symptoms of a neurocristopathy (Southard-Smith et al, 1998.…”

Section: Introductionmentioning

confidence: 99%

Neural crest cells retain their capability for multipotential differentiation even after lineage‐restricted stages

Motohashi

Yamanaka

Chiba

et al. 2011

Developmental Dynamics

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Multipotency of neural crest cells (NC cells) is thought to be a transient phase at the early stage of their generation; after NC cells emerge from the neural tube, they are specified into the lineage‐restricted precursors. We analyzed the differentiation of early‐stage NC‐like cells derived from Sox10‐IRES‐Venus ES cells, where the expression of Sox10 can be visualized with a fluorescent protein. Unexpectedly, both the Sox10+/Kit− cells and the Sox10+/Kit+ cells, which were restricted in vivo to the neuron (N)‐glial cell (G) lineage and melanocyte (M) lineage, respectively, generated N, G, and M, showing that they retain multipotency. We generated mice from the Sox10‐IRES‐Venus ES cells and analyzed the differentiation of their NC cells. Both the Sox10+/Kit− cells and Sox10+/Kit+ cells isolated from these mice formed colonies containing N, G, and M, showing that they are also multipotent. These findings suggest that NC cells retain multipotency even after the initial lineage‐restricted stages. Developmental Dynamics 240:1681–1693, 2011. © 2011 Wiley‐Liss, Inc.

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Development and degeneration of dorsal root ganglia in the absence of the HMG-domain transcription factor Sox10

Cited by 92 publications

References 60 publications

A zebrafish model for Waardenburg syndrome type IV reveals diverse roles for Sox10 in the otic vesicle

A zebrafish model for Waardenburg syndrome type IV reveals diverse roles for Sox10 in the otic vesicle

A direct role for Sox10 in specification of neural crest-derived sensory neurons

Neural crest cells retain their capability for multipotential differentiation even after lineage‐restricted stages

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