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Titolo:
Abnormalities in cerebellar Purkinje cells in the novel ataxic mutant mouse, pogo
Autore:
Jeong, YG; Hyun, BH; Hawkes, R;
Indirizzi:
Konyang Univ, Coll Med, Dept Anat, Nosan 320711, Chungnam, South Korea Konyang Univ Nosan Chungnam South Korea 320711 711, Chungnam, South Korea Korea Res Inst Biosci & Biotechnol, Genet Resource Ctr, Taejon 305333, South Korea Korea Res Inst Biosci & Biotechnol Taejon South Korea 305333 South Korea Univ Calgary, Fac Med, Dept Cell Biol & Anat, Calgary, AB T2N 4N1, Canada Univ Calgary Calgary AB Canada T2N 4N1 Anat, Calgary, AB T2N 4N1, Canada Univ Calgary, Fac Med, Genes & Dev Res Grp, Calgary, AB T2N 4N1, Canada Univ Calgary Calgary AB Canada T2N 4N1 s Grp, Calgary, AB T2N 4N1, Canada
Titolo Testata:
DEVELOPMENTAL BRAIN RESEARCH
fascicolo: 1-2, volume: 125, anno: 2000,
pagine: 61 - 67
SICI:
0165-3806(200012)125:1-2<61:AICPCI>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
NIEMANN-PICK DISEASE; STORAGE DISORDER; DEGENERATION; CHOLESTEROL;
Keywords:
immunocytochemistry; purkinje cell; ataxic mutant mouse;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
19
Recensione:
Indirizzi per estratti:
Indirizzo: Jeong, YG Konyang Univ, Coll Med, Dept Anat, Nosan 320711, Chungnam, SouthKorea Konyang Univ Nosan Chungnam South Korea 320711 nam, South Korea
Citazione:
Y.G. Jeong et al., "Abnormalities in cerebellar Purkinje cells in the novel ataxic mutant mouse, pogo", DEV BRAIN R, 125(1-2), 2000, pp. 61-67

Abstract

The pogo mouse is a novel neurological mutant, which was discovered, in aninbred strain (KJR/MsKist) derived from a Korean wild mouse. The pathological manifestation 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 toa failure of normal Purkinje cell activity. (C) 2000 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 24/09/20 alle ore 07:40:41