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Molecular and Cellular Biology, November 2002, p. 7398-7404, Vol. 22, No. 21
0270-7306/02/$04.00+0     DOI: 10.1128/MCB.22.21.7398-7404.2002
Copyright © 2002, American Society for Microbiology. All Rights Reserved.

Early Postnatal Death and Motor Disorders in Mice Congenitally Deficient in Calnexin Expression

Angela Denzel,1* Maurizio Molinari,2 Cesar Trigueros,1,{dagger} Joanne E. Martin,3 Shanti Velmurgan,3 Sue Brown,4 Gordon Stamp,5 and Michael J. Owen1,{ddagger}

Imperial Cancer Research Fund,1 Academic Department of Histopathology, Bart's and the Queen Mary's School of Medicine and Dentistry, Whitechapel,3 Neuromuscular Unit,4 Department of Histopathology, Imperial College, London, United Kingdom,5 Institute for Research in Biomedicine, Bellinzona, Switzerland2

Received 28 January 2002/ Returned for modification 18 March 2002/ Accepted 16 July 2002

Calnexin is a ubiquitously expressed type I membrane protein which is exclusively localized in the endoplasmic reticulum (ER). In mammalian cells, calnexin functions as a chaperone molecule and plays a key role in glycoprotein folding and quality control within the ER by interacting with folding intermediates via their monoglucosylated glycans. In order to gain more insight into the physiological roles of calnexin, we have generated calnexin gene-deficient mice. Despite its profound involvement in protein folding, calnexin is not essential for mammalian-cell viability in vivo: calnexin gene knockout mice were carried to full term, although 50% died within 48 h and the majority of the remaining mice had to be sacrificed within 4 weeks, with only a very few mice surviving to 3 months. Calnexin gene-deficient mice were smaller than their littermates and showed very obvious motor disorders, associated with a dramatic loss of large myelinated nerve fibers. Thus, the critical contribution of calnexin to mammalian physiology is tissue specific.

* Corresponding author. Present address: Department of Immunobiology, New Guy's House, Guy's, King's and St. Thomas's School of Medicine, London SE1 9RT, United Kingdom. Phone: 44 207 955 4976. Fax: 44 207 955 8894. E-mail: angela.denzel{at}kcl.ac.uk.

{dagger} Present address: Division of Immune Cell Biology, National Institute for Medical Research, London, United Kingdom.

{ddagger} Present address: Glaxosmithkline, Stevenage, United Kingdom.

Molecular and Cellular Biology, November 2002, p. 7398-7404, Vol. 22, No. 21
0022-538X/02/$04.00+0     DOI: 10.1128/MCB.22.21.7398-7404.2002
Copyright © 2002, American Society for Microbiology. All Rights Reserved.



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