Abnormalities in cerebellar Purkinje cells in the novel ataxic mutant mouse, pogo.
The pogo mouse is a novel neurological mutant, which was discovered, in an inbred strain (KJR/MsKist) derived from a Korean wild mouse. The pathological manifestations include difficulty in maintaining normal posture, failures of interlimb coordination and the inability to walk straight. The ataxia is first apparent from about 2 weeks of age and progresses throughout life. The mutation is inherited as an autosomal recessive trait. In this report, we describe abnormalities in the pogo/pogo cerebellum. Nissl staining shows that the pogo/pogo cerebellum is normal in size and lobulation. Similarly, immunocytochemical staining for a granule cell marker, 10B5, shows no differences in the thickness of the granular layer between pogo/pogo homozygote and pogo/+ heterozygote littermate controls. By using anti-parvalbumin immunocytochemistry, the cells of molecular layer of the pogo/pogo cerebellum also appeared similar in distribution as compared to normal wild type mouse. In anti-neurofilament immunocytochemistry, the basket cells axons of the pogo/pogo cerebellum appeared normal. Purkinje cell abnormalities were identified by using anti-calbindin D immunocytochemistry. In 120-day-old pogo/pogo mutant mice there was a loss of Purkinje cells throughout the cerebellar vermis. Furthermore, the somata and dendrites were extensively vacuolated in the pogo/pogo Purkinje cells and the primary dendrites were frequently swollen. Focal axonal swellings were commonly observed in the Purkinje cell axons of pogo/pogo mutant mice as they traversed the granular layer. These data suggest that the progressive ataxia seen in pogo mice may be due to a failure of normal Purkinje cell activity.
Jeong
Y G
YG
Department of Anatomy, College of Medicine, Konyang University, Nosan, 320-711, Chungnam, South Korea. ygjeong@kytis.konyang.ac.kr
Hyun
B H
BH
Hawkes
R
R
D000818:Animals; D000906:Antibodies; D064026:Calbindins; D002524:Cerebellar Ataxia / Q000235:genetics* / Q000473:pathology*; D002531:Cerebellum / Q000002:abnormalities; D003712:Dendrites / Q000737:chemistry / Q000473:pathology; D004195:Disease Models, Animal; D007150:Immunohistochemistry; D051379:Mice; D008818:Mice, Neurologic Mutants; D016900:Neurofilament Proteins / Q000032:analysis / Q000276:immunology; D009562:Nissl Bodies; D010320:Parvalbumins / Q000032:analysis / Q000276:immunology; D011689:Purkinje Cells / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D064030:S100 Calcium Binding Protein G / Q000032:analysis / Q000276:immunology
D000906:Antibodies; D064026:Calbindins; D016900:Neurofilament Proteins; D010320:Parvalbumins; D064030:S100 Calcium Binding Protein G