Scientific article

Calnexin deficiency leads to dysmyelination

Published inThe Journal of biological chemistry, vol. 285, no. 24, p. 18928-18938
Publication date2010

Calnexin is a molecular chaperone and a component of the quality control of the secretory pathway. We have generated calnexin gene-deficient mice (cnx(-/-)) and showed that calnexin deficiency leads to myelinopathy. Calnexin-deficient mice were viable with no discernible effects on other systems, including immune function, and instead they demonstrated dysmyelination as documented by reduced conductive velocity of nerve fibers and electron microscopy analysis of sciatic nerve and spinal cord. Myelin of the peripheral and central nervous systems of cnx(-/-) mice was disorganized and decompacted. There were no abnormalities in neuronal growth, no loss of neuronal fibers, and no change in fictive locomotor pattern in the absence of calnexin. This work reveals a previously unrecognized and important function of calnexin in myelination and provides new insights into the mechanisms responsible for myelin diseases.

  • Animals
  • Animals, Newborn
  • Calnexin/ genetics/metabolism/ physiology
  • Cell Membrane/metabolism
  • Demyelinating Diseases/ metabolism
  • Electrophysiology/methods
  • Endoplasmic Reticulum/metabolism
  • Female
  • Genotype
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Myelin Sheath/ metabolism
  • Protein Folding
  • Sciatic Nerve/metabolism/ultrastructure
  • Spinal Cord/metabolism/ultrastructure
Citation (ISO format)
KRAUS, Allison et al. Calnexin deficiency leads to dysmyelination. In: The Journal of biological chemistry, 2010, vol. 285, n° 24, p. 18928–18938. doi: 10.1074/jbc.M110.107201
Updates (1)
ISSN of the journal0021-9258

Technical informations

Creation08/27/2010 1:35:10 PM
First validation08/27/2010 1:35:10 PM
Update time03/14/2023 4:04:02 PM
Status update03/14/2023 4:04:02 PM
Last indexation10/18/2023 11:22:01 AM
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