PMID- 11598999
OWN - NLM
STAT- MEDLINE
DCOM- 20011204
LR  - 20231213
IS  - 0360-4012 (Print)
IS  - 0360-4012 (Linking)
VI  - 66
IP  - 1
DP  - 2001 Oct 1
TI  - Altered gene expression in Schwann cells of connexin32 knockout animals.
PG  - 23-36
AB  - The 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 gamma 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.
CI  - Copyright 2001 Wiley-Liss, Inc.
FAU - Nicholson, S M
AU  - Nicholson SM
AD  - Unite de Neurovirologie et Regeneration du Systeme Nerveux, Institut Pasteur, 
      Paris, France.
FAU - Gomes, D
AU  - Gomes D
FAU - de Nechaud, B
AU  - de Nechaud B
FAU - Bruzzone, R
AU  - Bruzzone R
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Neurosci Res
JT  - Journal of neuroscience research
JID - 7600111
RN  - 0 (Connexins)
RN  - 0 (DNA Primers)
RN  - 0 (Glial Fibrillary Acidic Protein)
SB  - IM
MH  - Animals
MH  - Cells, Cultured
MH  - Charcot-Marie-Tooth Disease/genetics/physiopathology
MH  - Connexins/*genetics
MH  - DNA Primers
MH  - Gene Expression/physiology
MH  - Glial Fibrillary Acidic Protein/genetics
MH  - Mice
MH  - Mice, Inbred C57BL
MH  - Mice, Knockout
MH  - Myelin Sheath/physiology
MH  - Phenotype
MH  - Schwann Cells/cytology/*physiology
MH  - Sciatic Nerve/cytology
MH  - Gap Junction beta-1 Protein
EDAT- 2001/10/13 10:00
MHDA- 2002/01/05 10:01
CRDT- 2001/10/13 10:00
PHST- 2001/10/13 10:00 [pubmed]
PHST- 2002/01/05 10:01 [medline]
PHST- 2001/10/13 10:00 [entrez]
AID - 10.1002/jnr.1194 [pii]
AID - 10.1002/jnr.1194 [doi]
PST - ppublish
SO  - J Neurosci Res. 2001 Oct 1;66(1):23-36. doi: 10.1002/jnr.1194.