title: Purkinje cell death: differences between developmental cell death and neurodegenerative death in mutant mice.
abstract: This review is devoted to Purkinje cell death occurring during development and in spontaneous cerebellar mutations of the mouse. We first present evidence in favor of an apoptotic developmental Purkinje cell death. Then, the different types of Purkinje cell degeneration occurring in mutant mice primarily affecting this neuronal population (nervous, purkinje cell degeneration, Lurcher, toppler, and woozy) are described and discussed. In addition, we show, by reporting new data, that cell death in tambaleante mutant mice can be related to autophagy. Last, we discuss the fact that the cell death pathways in mutant mice are more complex than the three types of developmental death generally described (apoptosis, autophagy, necrosis), since they share often characteristics of more than one type of these developmental cell deaths, particularly autophagy and apoptosis.
authors.affiliation: Université Pierre et Marie Curie-Paris 6, Paris, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Dusart
authors.forename: Jean Louis
authors.initials: JL
authors.lastname: Guenet
authors.forename: Constantino
authors.initials: C
authors.lastname: Sotelo
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000235:genetics*; D001343:Autophagy / Q000235:genetics; D002525:Cerebellar Cortex / Q000254:growth & development* / Q000473:pathology / Q000503:physiopathology*; D004195:Disease Models, Animal; D051379:Mice; D008818:Mice, Neurologic Mutants; D009336:Necrosis / Q000235:genetics; D009410:Nerve Degeneration / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D011689:Purkinje Cells / Q000473:pathology / Q000502:physiology*