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Calnexin deficiency leads to dysmyelination

Kraus, Allison
Groenendyk, Jody
Baldwin, T. A.
Dyck, Jason
Rosenbaum, E. E.
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Published in Journal of Biological Chemistry. 2010, vol. 285, no. 24, p. 18928-18938
Abstract 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.
Keywords AnimalsAnimals, NewbornCalnexin/ genetics/metabolism/ physiologyCell Membrane/metabolismDemyelinating Diseases/ metabolismElectrophysiology/methodsEndoplasmic Reticulum/metabolismFemaleGenotypeMaleMiceMice, Inbred C57BLMice, TransgenicMyelin Sheath/ metabolismProtein FoldingSciatic Nerve/metabolism/ultrastructureSpinal Cord/metabolism/ultrastructure
Stable URL http://archive-ouverte.unige.ch/unige:11385
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Other version: http://www.jbc.org/content/285/24/18928.full.pdf
PMID: 20400506
Research groups Radicaux libres et cellules souches embryonnaires (60)
Groupe Schaller Karl-Lothard (neurochirurgie) (851)
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