title: Calnexin deficiency leads to dysmyelination.
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.
authors.affiliation: Department of Biochemistry, School of Molecular and Systems Medicine, University of Alberta, Edmonton, Alberta T6G 2H7, Canada.
authors.forename: Allison
authors.initials: A
authors.lastname: Kraus
authors.forename: Jody
authors.initials: J
authors.lastname: Groenendyk
authors.forename: Karen
authors.initials: K
authors.lastname: Bedard
authors.forename: Troy A
authors.initials: TA
authors.lastname: Baldwin
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Krause
authors.forename: Michel
authors.initials: M
authors.lastname: Dubois-Dauphin
authors.forename: Jason
authors.initials: J
authors.lastname: Dyck
authors.forename: Erica E
authors.initials: EE
authors.lastname: Rosenbaum
authors.forename: Lawrence
authors.initials: L
authors.lastname: Korngut
authors.forename: Nansi J
authors.initials: NJ
authors.lastname: Colley
authors.forename: Simon
authors.initials: S
authors.lastname: Gosgnach
authors.forename: Douglas
authors.initials: D
authors.lastname: Zochodne
authors.forename: Kathryn
authors.initials: K
authors.lastname: Todd
authors.forename: Luis B
authors.initials: LB
authors.lastname: Agellon
authors.forename: Marek
authors.initials: M
authors.lastname: Michalak
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D037281:Calnexin / Q000235:genetics* / Q000378:metabolism / Q000502:physiology*; D002462:Cell Membrane / Q000378:metabolism; D003711:Demyelinating Diseases / Q000378:metabolism*; D004594:Electrophysiology / Q000379:methods; D004721:Endoplasmic Reticulum / Q000378:metabolism; D005260:Female; D005838:Genotype; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009186:Myelin Sheath / Q000378:metabolism*; D017510:Protein Folding; D012584:Sciatic Nerve / Q000378:metabolism / Q000648:ultrastructure; D013116:Spinal Cord / Q000378:metabolism / Q000648:ultrastructure
chemical_list: D037281:Calnexin
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