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          Institute: MPI für Neurobiologie     Collection: Molecular Neurobiology     Display Documents



  history
ID: 477021.0, MPI für Neurobiologie / Molecular Neurobiology
Sox10 is required for Schwann cell identity and progression beyond the immature Schwann cell stage
Authors:Finzsch, M.; Schreiner, S.; Kichko, T.; Reeh, P.; Tamm, E. R.; Bösl, M. R.; Meijer, D.; Wegner, M.
Language:English
Date of Publication (YYYY-MM-DD):2010-05-17
Title of Journal:Journal of Cell Biology
Journal Abbrev.:J. Cell Biol.
Volume:189
Issue / Number:4
Start Page:701
End Page:712
Review Status:Peer-review
Audience:Not Specified
Abstract / Description:Mutations in the transcription factor SOX10 cause neurocristopathies, including Waardenburg-Hirschsprung syndrome and peripheral neuropathies in humans. This is partly attributed to a requirement for Sox10 in early neural crest for survival, maintenance of pluripotency, and specification to several cell lineages, including peripheral glia. As a consequence, peripheral glia are absent in Sox10-deficient mice. Intriguingly, Sox10 continues to be expressed in these cells after specification. To analyze glial functions after specification, we specifically deleted Sox10 in immature Schwann cells by conditional mutagenesis. Mutant mice died from peripheral neuropathy before the seventh postnatal week. Nerve alterations included a thinned perineurial sheath, increased lipid and collagen deposition, and a dramatically altered cellular composition. Nerve conduction was also grossly aberrant, and neither myelinating nor non-myelinating Schwann cells formed. Instead, axons of different sizes remained unsorted in large bundles. Schwann cells failed to develop beyond the immature stage and were unable to maintain identity. Thus, our study identifies a novel cause for peripheral neuropathies in patients with SOX10 mutations.
External Publication Status:published
Document Type:Article
Version Comment:Automatic journal name synchronization
Affiliations:MPI für Neurobiologie/Molecular Neurobiology (Klein)
External Affiliations:[Finzsch, Markus; Schreiner, Silke; Wegner, Michael] Univ Erlangen Nurnberg, Inst Biochem, Emil Fischer Zentrum, D-91054 Erlangen, Germany.; [Kichko, Tatjana; Reeh, Peter] Univ Erlangen Nurnberg, Inst Physiol & Pathophysiol, D-91054 Erlangen, Germany.; [Tamm, Ernst R.] Univ Regensburg, Inst Humananat & Embryol, D-93053 Regensburg, Germany.; [Meijer, Dies] Erasmus MC, Dept Cell Biol & Genet, NL-3015 CE Rotterdam, Netherlands.
Identifiers:ISI:000277777700011 [ID No:1]
ISSN:0021-9525 [ID No:2]
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