Summary
Inherited dystrophy of the retina in young inbred C3H mice, which were all fully afflicted, has been studied in tissue cultures in order to examine the pathogenesis and to observe the pattern of development of the disease. Expiants of retinal tissue both from C3H and normal mice grew in vitro to become mono-layer cultures consisting of differentiated nerve cells, visual cells, neuroglial cells and pigment epithelium cells on the surface of a monolayer of cells of mesenchymal origin. Evidence has been obtained indicating that the progressive degeneration of the rods depends upon a primary disturbance of the cell metabolism. The visual cells from young C3H mice formed rosettes with a central lumen bordered by a membranous structure and rod-like process shorter than those in cultures from normal mice. All the rods started to degenerate at about the same time in vitro and in vivo although the progress to complete destruction proceeded faster in tissue cultures than in vivo. The nerve cells, neuroglial cells, pigment epithelium cells and cells of mesenchymal origin from C3H mice showed no signs of such a sudden spontaneous degeneration, and no other differences as compared to the retinal cultures from normal mice. There was only a slight increase in the number of phagocytes and mast cells during the degeneration of the rods from C3H mice. Large, multipolar cells, resembling hypertrophie, re-active astrocytes, have been observed only in cultures from C3H mice with disintegrated rods.
Zusammenfassung
Explantate von Netzhaut sowohl der kranken C3H-Mäuse wie normaler Mäuse wachsen in vitro über einer flächenhaften Lage (monolayer) von mesenchymalen Zellen in Form einer flachen Zellage, die aus differenzierten Nervenzellen, Sehzellen, Neuroglialzellen, Pigmentepithelien und Zellen mesenchymaler Herkunft besteht. Die dabei zu erhebenden Befunde weisen darauf hin, daß die fortschreitende Degeneration der Stäbchen bei den erkrankten Tieren abhängig ist von einer primären Störung des Zellstoffwechsels. Die Sehzellen von jungen C3H-Mäusen bilden Rosetten mit einer zentralen Lichtung, die von einer membran-artigen Struktur und Stäbchenfortsätzen begrenzt ist; diese letzteren sind aber kürzer als diejenigen normaler Mäuse. Alle Stäbchen beginnen etwa zur selben Zeit in vivo und in vitro zu degenerieren, allerdings läuft dieser Vorgang in der Gewebekultur schneller ab als am lebenden Tier. Die Nervenzellen, Neuroglia-zellen, Pigmentepithelien und Zellen mesenchymaler Herkunft der C3H-Mäuse zeigen keine Zeichen einer solchen plötzlichen spontanen Degeneration und auch keine anderen Unterschiede gegenüber den Netzhautkulturen normaler Mäuse. Während der Degeneration der Stäbchen bei den C3H-Mäusen findet sich eine leichte Vermehrung der Zahl der Phagocyten und Mastzellen. Große multipolare Zellen, welche hypertrophischen reaktiven Astrocyten gleichen, konnten nur in den Kulturen von C3H-Mäusen mit zerfallenden Stäbchen beobachtet werden.
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This study was supported by a grant from the University of Göteborg and a grant to Assoc. Prof. P. Sourander, Y 184, from the Swedish Medical Research Council.
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Hansson, H.A. A tissue culture study of inherited dystrophy of the retina in mice. Virchows Arch. path Anat. 340, 69–83 (1965). https://doi.org/10.1007/BF00960252
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DOI: https://doi.org/10.1007/BF00960252