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Early Postnatal Death and Motor Disorders in Mice Congenitally Deficient in Calnexin Expression
- Source :
- Molecular and Cellular Biology. 22:7398-7404
- Publication Year :
- 2002
- Publisher :
- Informa UK Limited, 2002.
-
Abstract
- 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.
- Subjects :
- Heterozygote
Protein Folding
Glycan
Time Factors
Calnexin
Cell Survival
Endoplasmic Reticulum
Transfection
Nerve Fibers, Myelinated
Mice
Mammalian Genetic Models with Minimal or Complex Phenotypes
Animals
Molecular Biology
Alleles
Myelin Sheath
Gene knockout
Mice, Knockout
Motor Neurons
Recombination, Genetic
chemistry.chemical_classification
Models, Genetic
biology
Stem Cells
Endoplasmic reticulum
Cell Biology
Embryo, Mammalian
Molecular biology
Cell biology
Mice, Inbred C57BL
Microscopy, Electron
Phenotype
Animals, Newborn
chemistry
Membrane protein
Chaperone (protein)
biology.protein
Protein folding
Glycoprotein
Molecular Chaperones
Subjects
Details
- ISSN :
- 10985549
- Volume :
- 22
- Database :
- OpenAIRE
- Journal :
- Molecular and Cellular Biology
- Accession number :
- edsair.doi.dedup.....7d767212995671e05e0af0e7be3de948