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Article: Altered gene expression in Schwann cells of connexin32 knockout animals

TitleAltered gene expression in Schwann cells of connexin32 knockout animals
Authors
KeywordsCharcot-Marie-Tooth
Cx32
GFAP
Myelin
PNS
Schwann cells
Issue Date2001
PublisherJohn Wiley & Sons, Inc. The Journal's web site is located at http://www3.interscience.wiley.com/cgi-bin/jhome/34828
Citation
Journal Of Neuroscience Research, 2001, v. 66 n. 1, p. 23-36 How to Cite?
AbstractThe discovery that the dominant X-linked form of Charcot-Marie-Tooth disease (CMTX), a genetic disease of the peripheral nervous system (PNS), is associated with mutations in connexin32 (Cx32) has brought attention to the importance of connexins in glial cell biology. To gain further insight into the consequences of Cx32 deficiency, we have undertaken a detailed characterization of the gene expression profile of Schwann cells isolated from the sciatic nerve of wild-type and Cx32-null mice. Schwann cells exhibit two distinct phenotypes, myelinating and nonmyelinating, which are defined by their different morphology with respect to axons and by their unique profile of gene expression. Our findings show that, regardless of the mouse genotype, cultured Schwann cells express similar levels of messages for a number of connexins and for genes characteristic of both the myelinating and the nonmyelinating phenotypes. Furthermore, we have identified Cx36, a member of the γ subclass of connexins, which are preferentially expressed in neuronal cells of mouse brain and retina, as an additional connexin present in Schwann cells. Mice lacking Cx32, however, exhibited a marked up-regulation of glial fibrillary acidic protein (GFAP), a cytoskeletal protein usually synthesized only by nonmyelinating Schwann cells. This observation was extended to the PNS in vivo and did not reflect a general perturbation of the expression of other nonmyelinating Schwann cell genes. These findings demonstrate that the absence of Cx32 results in a distinct pattern of gene dysregulation in Schwann cells and that Schwann cell homeostasis is critically dependent on the correct expression of Cx32 and not just any connexin. Identifying the relationship between increased GFAP expression and the absence of Cx32 could lead to the definition of specific roles for Cx32 in the control of myelin homeostasis and in the development of CMTX. J. Neurosci. Res. © 2001 Wiley-Liss, Inc.
Persistent Identifierhttp://hdl.handle.net/10722/132715
ISSN
2015 Impact Factor: 2.689
2015 SCImago Journal Rankings: 1.261
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorNicholson, SMen_HK
dc.contributor.authorGoms, Den_HK
dc.contributor.authorDe Nchaud, Ben_HK
dc.contributor.authorBruzzone, Ren_HK
dc.date.accessioned2011-03-28T09:28:29Z-
dc.date.available2011-03-28T09:28:29Z-
dc.date.issued2001en_HK
dc.identifier.citationJournal Of Neuroscience Research, 2001, v. 66 n. 1, p. 23-36en_HK
dc.identifier.issn0360-4012en_HK
dc.identifier.urihttp://hdl.handle.net/10722/132715-
dc.description.abstractThe discovery that the dominant X-linked form of Charcot-Marie-Tooth disease (CMTX), a genetic disease of the peripheral nervous system (PNS), is associated with mutations in connexin32 (Cx32) has brought attention to the importance of connexins in glial cell biology. To gain further insight into the consequences of Cx32 deficiency, we have undertaken a detailed characterization of the gene expression profile of Schwann cells isolated from the sciatic nerve of wild-type and Cx32-null mice. Schwann cells exhibit two distinct phenotypes, myelinating and nonmyelinating, which are defined by their different morphology with respect to axons and by their unique profile of gene expression. Our findings show that, regardless of the mouse genotype, cultured Schwann cells express similar levels of messages for a number of connexins and for genes characteristic of both the myelinating and the nonmyelinating phenotypes. Furthermore, we have identified Cx36, a member of the γ subclass of connexins, which are preferentially expressed in neuronal cells of mouse brain and retina, as an additional connexin present in Schwann cells. Mice lacking Cx32, however, exhibited a marked up-regulation of glial fibrillary acidic protein (GFAP), a cytoskeletal protein usually synthesized only by nonmyelinating Schwann cells. This observation was extended to the PNS in vivo and did not reflect a general perturbation of the expression of other nonmyelinating Schwann cell genes. These findings demonstrate that the absence of Cx32 results in a distinct pattern of gene dysregulation in Schwann cells and that Schwann cell homeostasis is critically dependent on the correct expression of Cx32 and not just any connexin. Identifying the relationship between increased GFAP expression and the absence of Cx32 could lead to the definition of specific roles for Cx32 in the control of myelin homeostasis and in the development of CMTX. J. Neurosci. Res. © 2001 Wiley-Liss, Inc.en_HK
dc.languageengen_US
dc.publisherJohn Wiley & Sons, Inc. The Journal's web site is located at http://www3.interscience.wiley.com/cgi-bin/jhome/34828en_HK
dc.relation.ispartofJournal of Neuroscience Researchen_HK
dc.subjectCharcot-Marie-Toothen_HK
dc.subjectCx32en_HK
dc.subjectGFAPen_HK
dc.subjectMyelinen_HK
dc.subjectPNSen_HK
dc.subjectSchwann cellsen_HK
dc.titleAltered gene expression in Schwann cells of connexin32 knockout animalsen_HK
dc.typeArticleen_HK
dc.identifier.emailBruzzone, R: bruzzone@hkucc.hku.hken_HK
dc.identifier.authorityBruzzone, R=rp01442en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1002/jnr.1194en_HK
dc.identifier.pmid11598999-
dc.identifier.scopuseid_2-s2.0-0035477457en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0035477457&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume66en_HK
dc.identifier.issue1en_HK
dc.identifier.spage23en_HK
dc.identifier.epage36en_HK
dc.identifier.isiWOS:000171297900004-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridNicholson, SM=36950172200en_HK
dc.identifier.scopusauthoridGoms, D=23106172200en_HK
dc.identifier.scopusauthoridDe Nchaud, B=23105601300en_HK
dc.identifier.scopusauthoridBruzzone, R=7006793327en_HK

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