Article
Rabbit paralytic tremor phenotype--a plp1 gene mutation as a model of human Pelizaeus-Merzbacher disease.
Acta neurobiologiae experimentalis - 1 Jan 2005
Sypecka Joanna, Domańska-Janik Krystyna
Abstract excerpt
The paralytic tremor (pt) disease in rabbits results from a point mutation in a plp gene and manifests itself by a broad range of neurological signs. Biochemical studies have shown that myelinogenesis is retarded and deficient in mutant rabbits. Myelin sheaths are usually thin and malformed. The number of oligodendrocytes is normal, however their differentiation and maturation is prolonged. The effects of the pt...
Topics
- Animals
- Demyelinating Diseases
- Disease Models, Animal
- Humans
- Membrane Proteins
- Myelin Proteolipid Protein
- Paresis
- Pelizaeus-Merzbacher Disease
- Phenotype
- Point Mutation
- Rabbits
- Tremor
