| First Author | Ghetti B | Year | 1978 |
| Journal | J Neuropathol Exp Neurol | Volume | 37 |
| Pages | 617 (Abstr 109) | Mgi Jnum | J:28476 |
| Mgi Id | MGI:76085 | Citation | Ghetti B, et al. (1978) Studies on the Purkinje Cell degeneration (pcd) mutant: Primary pathology and transneuronal changes. J Neuropathol Exp Neurol 37:617 (Abstr 109) |
| abstractText | Full text of Abstract: 109 STUDIES OH THE PURKINJE CELL DEGENERATION (pcd) MUTANT: PRIMARY PATHOLOGY AND TRANSNEURONAL CHANGES. B. Ghetti,* C.J. Alyea and J. Muller. Indiana University School of Medicine, Indianapolis, Indiana. Purkinje cell degeneration (pcd) is an autosomal recessive murine mutation leading to degeneration and loss of the Purkinje cells (Pc) (Mullen et al.). Cytological changes become manifest at postnatal day 15, when the PC synaptogenesis is complete; the mutants can be detected clinically at day 22, by the ataxia. In mutants aged 15 days to one year we have studied with light and electron microscopy: 1) the early changes and the pattern of degeneration of the Pc, 2) the transneuronal changes secondary to Pc loss. Between day 15 and 18 the Pc perikaryon shows fewer cisterns of endoplasmic reticulum (ER) and a large polysomal mass. From day 18 progressive darkening of the cytoplasm and shrinkage of the Pc takes place, followed by fragmentation and removal of Pc debris. Between day 20-27 PC dendrites show focal dilatation of the agranular ER and microtubule loss; their cytoplasm is floccular and osmiophilic in the late stage of degeneration. Similar changes can be observed in the Pc axon and terminal boutons. Following removal of the bulk of the Pc dendrites, osmiophilic debris, postsynaptic to parallel and climbing fibers, can be found in 3 to 6 month olds; the majority of boutons are denuded of their postsynaptic targets. Between 3-6 months numerous granule cells and processes undergo dark degeneration. Between 6-12 months the granule cell layer is severely depleted. Mossy fiber endings have lost their postsynaptic element and are surrounded by astrocytic processes. In the deafferentated deep cerebellar nuclei nerve cells are more densely packed than in controls. |
