Altered 9-O acetylation of disialogangliosides in cerebellar Purkinje cells of the nervous mutant mouse

Purkinje cells are critical for the function of cerebellum. The degeneration of Purkinje cells leads to defects in motion control. We have found that Purkinje cells specifically express Ankfy1 protein during development and in adult. This protein seems to play minor functions during development as Ankfy1 knockout mice appear normal till adult. However, at 9-month-old, knockout mice showed abnormal cerebellum with reduced vermis size and developed defective motor function. Further investigation demonstrated that the cerebellum of the mutant mouse has lost most of its Purkinje cells, while other cerebellar cells remained largely normal. Our data suggested that the Ankfy1 might be important for the maintenance of cerebellar Purkinje cells.

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Abnormalities in cerebellar Purkinje cells in the novel ataxic mutant mouse, pogo

Developmental Brain Research ◽

10.1016/s0165-3806(00)00114-0 ◽

2000 ◽

Vol 125 (1-2) ◽

pp. 61-67 ◽

Cited By ~ 23

Author(s):

Young-Gil Jeong ◽

Byung-Hwa Hyun ◽

Richard Hawkes

Keyword(s):

Purkinje Cells ◽

Mutant Mouse ◽

The Novel ◽

Cerebellar Purkinje Cells

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Dissection of the Cellular and Molecular Events that Position Cerebellar Purkinje Cells: A Study of themath1Null-Mutant Mouse

Journal of Neuroscience ◽

10.1523/jneurosci.22-18-08110.2002 ◽

2002 ◽

Vol 22 (18) ◽

pp. 8110-8116 ◽

Cited By ~ 39

Author(s):

Patricia Jensen ◽

Huda Y. Zoghbi ◽

Dan Goldowitz

Keyword(s):

Purkinje Cells ◽

Mutant Mouse ◽

Molecular Events ◽

Cerebellar Purkinje Cells

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Ultrastorcture of Cerebellar Purkinje Cells in Rats Subjected To Partial Global Cerebral Ischemia

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100120060 ◽

1985 ◽

Vol 43 ◽

pp. 674-675

Author(s):

R.V.W. Dimlich ◽

M.H. Biros

Keyword(s):

Cerebral Ischemia ◽

Purkinje Cells ◽

Cell Types ◽

Microscopic Level ◽

Global Cerebral Ischemia ◽

Light Microscopic Level ◽

Cerebellar Damage ◽

Cerebellar Injury ◽

Cerebellar Purkinje Cells ◽

Cell Changes

In severe cerebral ischemia, Purkinje cells of the cerebellum are one of the cell types most vulnerable to anoxic damage. In the partial (forebrain) global ischemic (PGI) model of the rat, Paljärvi noted at the light microscopic level that cerebellar damage is inconsistant and when present, milder than in the telencephalon, diencephalon and rostral brain stem. Cerebellar injury was observed in 3 of 4 PGI rats following 5 minutes of reperfusion but in none of the rats after 90 min of reperfusion. To evaluate a time between these two extremes (5 and 90 min), the present investigation used the PGI model to study the effects of ischemia on the ultrastructure of cerebellar Purkinje cells in rats that were sacrificed after 30 min of reperfusion. This time also was chosen because lactic acid that is thought to contribute to ischemic cell changes in PGI is at a maximum after 30 min of reperfusion.

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