Article
Myelin proteolipid protein mutation in the rabbit: a new model of Pelizaeus-Merzbacher disease.
Schweizer Archiv fur Neurologie und Psychiatrie (Zurich, Switzerland : 1985) - 1 Jan 1994
Tosic M, Dolivo M, Domanska-Janik K, Matthieu J M
Abstract excerpt
Proteolipid protein (PLP) is a major myelin protein of the central nervous system. Mutations of the Plp gene are responsible for a number of sex-linked disorders in humans (Pelizaeus-Merzbacher disease) and in animals. We have identified a novel mutation of the Plp gene which gives rise to the paralytic tremor (pt) phenotype in rabbit. Pt rabbits are hypomyelinated and present very low levels of PLP protein and...
Topics
- Animals
- Diffuse Cerebral Sclerosis of Schilder
- Disease Models, Animal
- Genotype
- Mutation
- Myelin Proteins
- Myelin Proteolipid Protein
- Phenotype
- RNA, Messenger
- Rabbits