Cell death of oligodendrocytes or demyelination induced by overexpression of proteolipid protein depending on expressed gene dosage.
The transgenic mice, which were produced by introducing the wild type proteolipid protein (PLP) gene, revealed different neurological symptoms depending on expressed gene dosage. Homozygotes, which bore four more copies of the extra PLP gene, died before 4 weeks old with severe tremors and convulsions, while histologically hypomyelination and death of the oligodendrocytes were particularly noticeable. Heterozygotes, which bore two more copies and survived nearly 1 year, suffered from severe hindlimb palsy and bladder-rectal disturbances before death. These symptoms probably resulted from striking demyelination which occurred abruptly at a later stage of life. The heterozygous PLP-transgenic mice are thus available for a model of demyelination diseases.
Inoue
Y
Y
Department of Anatomy, Hokkaido University School of Medicine, Sapporo, Japan. inoyoshi@med.hokudai.ac.jp
Kagawa
T
T
Matsumura
Y
Y
Ikenaka
K
K
Mikoshiba
K
K
D000818:Animals; D016923:Cell Death; D003711:Demyelinating Diseases / Q000235:genetics*; D018628:Gene Dosage; D051379:Mice; D008822:Mice, Transgenic; D018991:Myelin Proteolipid Protein / Q000235:genetics*; D009836:Oligodendroglia / Q000473:pathology* / Q000502:physiology; D009900:Optic Nerve / Q000473:pathology* / Q000502:physiology; D013116:Spinal Cord / Q000473:pathology
D018991:Myelin Proteolipid Protein