RGD Reference Report - Familial dysmyelination in a Long Evans rat mutant. - Rat Genome Database

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Familial dysmyelination in a Long Evans rat mutant.

Authors: Delaney, KH  Kwiecien, JM  Wegiel, J  Wisniewski, HM  Percy, DH  Fletch, AL 
Citation: Delaney KH, etal., Lab Anim Sci 1995 Oct;45(5):547-53.
RGD ID: 1358775
Pubmed: PMID:8569154   (View Abstract at PubMed)

Tremors were observed in 15 Long Evans rats beginning at 10 to 12 days of age. These were followed by progressively worsening ataxia, hind limb paresis, episodes of immobility, and seizures by 5 to 14 weeks. Gross lesions were not observed at necropsy in rats euthanized and perfused at 4 to 16 weeks of age. Neurohistologic examination revealed dysmyelination in the central nervous system. Astrogliosis in the white matter with marked increase of expression of the glial fibrillary acid protein marker was accompanied by diffuse microgliosis. Scattered glial cells, interpreted to be oligodendrocytes, contained minute periodic acid-Schiff-positive cytoplasmic granules. Large mineralized periodic acid-Schiff-positive and laminated structures were observed in the cerebellar white matter, midbrain, and thalamus of rats over 6 weeks old. Neuronal degeneration and loss was evident in the cortex, hippocampus, and midbrain. Large axonal spheroids were found in the ventral and lateral funiculi of the spinal cord. An ultrastructural study of four affected rats revealed an almost complete absence of myelinated axons and normal sheaths, and degeneration and necrosis of oligodendrocytes. The Long Evans shaker rat represents a novel myelin mutant with a remarkable survival period and appears to have an autosomal recessive mode of inheritance.

Phenotype Annotations    Click to see Annotation Detail View

Mammalian Phenotype

TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
ataxia  IAGP 1358775 RGD 
dysmyelination  IAGP 1358775 RGD 
tremors  IAGP 1358775 RGD 
Objects Annotated

Strains
LE-Mbpmd  (NA)

Objects referenced in this article
Gene Mbp myelin basic protein Rattus norvegicus

Additional Information