Document Detail

Striking pattern of Purkinje cell loss in cerebellum of an ataxic mutant mouse, tottering.
MedLine Citation:
PMID:  19325647     Owner:  NLM     Status:  MEDLINE    
Tottering mouse is an ataxic mutant that carries a mutation in a gene encoding for the apha1A subunit of P/Q-type Ca2+ channel (Cav2.1). This study revisited to examine whether a Purkinje cell loss occurred in the cerebellum of tottering mice. In tottering mice, Calbindin D-28k negative gaps were apparent in the vermis but not in the hemisphere. Calbindin D-28k immunofluorescence with DAPI staining demonstrated the absence of Purkinje cells in the Calbindin D-28k negative gaps. The Purkinje cell loss seemed to be observed prominently in the zebrin II negative compartments of the anterior vermis, but in the zebrin II positive compartments of the posterior vermis. Quite consistent with the histopathological observations, quantitation of the density of Calbindin D-28k and zebrin II immunopositive Purkinje cells in the tottering cerebellum revealed that the Purkinje cells were selectively lost in the zebrin II immunonegative compartments of the lobules I and II but in the zebrin II immunopositive compartments in the lobule IX. Those results predict that the susceptibility to the Cav2.1 gene defect is different among Purkinje cell phenotypes of the tottering cerebellum rather than the expression pattern of mutated Cav2.1 channels. This may result in the reproducible parasagittal pattern of Purkinje cell loss.
Kazuhiko Sawada; Abul Kalam Azad; Hiromi Sakata-Haga; Nam-Seob Lee; Young-Gil Jeong; Yoshihiro Fukui
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta neurobiologiae experimentalis     Volume:  69     ISSN:  0065-1400     ISO Abbreviation:  Acta Neurobiol Exp (Wars)     Publication Date:  2009  
Date Detail:
Created Date:  2009-03-27     Completed Date:  2009-05-18     Revised Date:  2013-07-03    
Medline Journal Info:
Nlm Unique ID:  1246675     Medline TA:  Acta Neurobiol Exp (Wars)     Country:  Poland    
Other Details:
Languages:  eng     Pagination:  138-45     Citation Subset:  IM    
Department of Anatomy and Developmental Neurobiology, University of Tokushima Graduate School Institute of Health Biosciences, Tokushima, Japan.
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MeSH Terms
Ataxia / genetics,  pathology*
Calcium Channels, P-Type / genetics
Calcium Channels, Q-Type / genetics
Calcium-Binding Protein, Vitamin D-Dependent / metabolism
Cell Count / methods
Cell Death / genetics
Cerebellum / pathology*
Indoles / diagnostic use
Mice, Inbred C57BL
Mice, Neurologic Mutants
Nerve Tissue Proteins / metabolism
Organ Size / genetics
Purkinje Cells / metabolism,  pathology*
Reg. No./Substance:
0/Calcium Channels, P-Type; 0/Calcium Channels, Q-Type; 0/Calcium-Binding Protein, Vitamin D-Dependent; 0/Indoles; 0/Nerve Tissue Proteins; 0/calbindin; 0/voltage-dependent calcium channel (P-Q type); 0/zebrin II; 47165-04-8/DAPI

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