Premium
Development of Cerebellar Hypoplasia in Jaundiced Gunn Rats: A Morphological Study
Author(s) -
TAKAGISHI Yoshiko
Publication year - 1989
Publication title -
congenital anomalies
Language(s) - English
Resource type - Journals
eISSN - 1741-4520
pISSN - 0914-3505
DOI - 10.1111/j.1741-4520.1989.tb00749.x
Subject(s) - granular layer , purkinje cell , cerebellum , golgi apparatus , cerebellar cortex , endoplasmic reticulum , cytoplasm , ultrastructure , anatomy , biology , electron microscope , cerebellar hypoplasia (non human) , pathology , chemistry , microbiology and biotechnology , neuroscience , medicine , physics , optics
The developmental process of cerebellar hypoplasia was investigated in homozygous (j/j) Gunn rats (born from heterozygous (+/j) mothers) with hereditary hyperbilirubinemia by light microscopy of Epon‐embedded sections and Golgi preparations, electron microscopy and immunofluorescence with anti‐SlOO protein immune serum. No abnormalities were found in the cerebellar cortex at postnatal day 1. But at day 3, some Purkinje cells contained whorled membranous inclusions, enlarged Golgi cisternae and vesicles, and/or dilated endoplasmic reticulum. Membranous inclusions filled the cytoplasm of a large number of Purkinje cells at day 10. These severely damaged Purkinje cells died and disappeared by day 30. The remaining Purkinje cells contained only a few membranous inclusions at day 30. At the adult stage, membranous inclusions were no longer detected in the remaining Purkinje cells. In these Purkinje cells, the dendrites were poorly developed and primitive synaptic junctions with parallel fibers were occasionally found on the dendritic shafts even at the adult stage. The perisomatic processes were often present on the remaining Purkinje cell soma from day 18 to the adult stage and were in synaptic contact with climbing fibers. The external granular layer was thinner in j/j than in +/j rats without hyperbilirubinemia at days 12 and 15 and disappeared at day 18 in j/j rats, i.e., earlier in j/j than in +/j rats. The molecular layer was thinner at day 10 and became slightly thicker thereafter. Cells in the molecular layer were scarce after day 12. The internal granular layer was thinner after day 12. An insufficient number of cells in the internal granular layer was apparent after day 12. Bergmann glia cells were hypertrophied after day 10 and filledthe spaces where Purkinje cells had disappeared. Subsequently, Bergmann fibers and astrocytes in the internal granular layer were hypertrophied after day 15. Thus, in the developmental process of cerebellar hypoplasia in j/j Gunn rats, Purkinje cell damage preceded the histogenetic abnormalities of the cortex including astrocytic changes.