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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 76 (1988), S. 366-372 
    ISSN: 1432-0533
    Keywords: Cortical dysplasia ; Neuronal migration disorder ; Neurological mutant ; Shaking rat ; Kawasaki (SRK) ; Wistar rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Shaking rat Kawasaki (SRK), a newly discovered neurological mutant rat in the Wistar strain, is described. The abnormalities of SRK rats are transmitted as an autosomal recessive trait. The neurological signs are shaking of the body and an ataxic-paretic gait from day 10 postnatal. The affected rats survive for about 1 month. Macroscopically, the cerebellum is small and frequently the vermis and paraflocculus lacking. The most conspicuous histological finding in the central nervous system is malposition of the neurons in the cerebral cortex, hippocampus and cerebellum. Myelination and synapse formation are intact. Abnormal myelinated fibers are present in the molecular layer of the cerebral cortex and in the central gray matter of the spinal cord. These morphological abnormalities resemble those reported in the reeler mutant mouse. SRK rats are another good animal model of human congenital malformations with neuronal migration disorders.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Mitochondrial myopathy ; Cytochromec oxidase ; Complex IV ; Muscle histochemistry ; Ragged-red fibers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Muscle biopsies from 16 patients with cytochromec oxidase (CCO) deficiency were examined morphologically. Two siblings had the fatal infantile form. The muscle of the older sister at the age of 5 months had numerous ragged-red fibers (RRF) and increased numbers of lipid droplets; at 28 days the brother had no RRF suggesting that the RRF formed later than 28 days. The muscle pathology in two patients with the benign infantile form improved as they grew older; numbers of RRF, lipid droplets and glycogen particles decreased and CCO activity increased in the second biopsy. In the encephalomyopathic form, RRF were seen in 5 of 12 muscles mostly in patients more than 6 years of age. Muscle spindles and blood vessel walls in the biopsies from three patients with rapid clinical aggravation had no CCO activity, suggesting that enzyme activity differed from tissue to tissue (tissue specificity).
    Type of Medium: Electronic Resource
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