Article
Modulation of rumpshaker phenotype with wild-type PLP/DM20 suggests several pathogenic mechanisms.
Journal of neuroscience research - 1 Aug 2010
Barrie Jennifer A, Montague Paul, Karim Saadia, Kirkham Douglas, Nave Klaus-Armin, Anderson Thomas J, Griffiths Ian R, McLaughlin Mark
Abstract excerpt
The rumpshaker mutation of the murine myelin proteolipid protein 1 (Plp1) gene generates misfolded PLP/DM20 protein, resulting in dysmyelination, increased oligodendrocyte apoptosis, and death prior to P40 when expressed on the C57 BL/6 background. In this study, we used transgenic complementation to normalize the levels of PLP/DM20 in myelin with wild-type protein to determine whether loss of normal PLP function...
Topics
- Aging
- Animals
- Apoptosis
- Astrocytes
- DNA-Binding Proteins
- Endoplasmic Reticulum Chaperone BiP
- Heat-Shock Proteins
- Male
- Mice
- Mice, Inbred C57BL
- Mice, Transgenic