Document Detail


Progressive atrophy of cerebellar Purkinje cell dendrites during aging of the heterozygous staggerer mouse (Rora(+/sg)).
MedLine Citation:
PMID:  11248354     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Staggerer (Rora(sg/sg)) is an autosomal mutation in an orphan nuclear hormone receptor gene, RORalpha, that acts intrinsically within the Purkinje cells and causes dysgenesis of the cerebellar cortex. Purkinje cell number is severely reduced, and the surviving cells are small with poorly developed dendrites. In contrast, the cytoarchitecture of the cerebellar cortex of the heterozygous staggerer (Rora(+/sg)) appears to be normal. However, quantitative studies have revealed a premature loss of Purkinje cells with advancing age. Most of the loss (25--30%) is complete by 13 months with little change thereafter. To address the question of whether all Purkinje cells, even the surviving ones, are affected by aging even though their cell bodies remain intact, we studied the evolution with age of the dendritic arbor through a semi-quantitative analysis of Golgi-impregnated Purkinje cells. A total of ten different morphological parameters were measured in 4-, 12- and 22-month-old wild type and heterozygous Rora(+/sg) mice. While the effects of the aging process are apparent in the wild type cerebellum, they are considerably accelerated in the Rora(+/sg) mouse. By 12 months the Rora(+/sg) Purkinje cell dendrite is as atrophic as a wild type dendrite from a 22-month-old and the dendritic regression continues well beyond the period of cell death in the heterozygous Rora(+/sg) mouse.
Authors:
N Hadj-Sahraoui; F Frederic; H Zanjani; N Delhaye-Bouchaud; K Herrup; J Mariani
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Brain research. Developmental brain research     Volume:  126     ISSN:  0165-3806     ISO Abbreviation:  Brain Res. Dev. Brain Res.     Publication Date:  2001 Feb 
Date Detail:
Created Date:  2001-03-15     Completed Date:  2001-05-24     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  8908639     Medline TA:  Brain Res Dev Brain Res     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  201-9     Citation Subset:  IM    
Affiliation:
Laboratoire Développement et Vieillissement du Système Nerveux, Boite 14, Institut des Neurosciences, UMR 7624 CNRS, Université P. & M. Curie, 9 quai Saint Bernard, 75005 Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Aging / genetics,  pathology*
Animals
Atrophy / genetics,  pathology
Cell Death / genetics
Cerebellar Diseases / genetics,  pathology*
Cerebellum / pathology*
Dendrites / pathology*
Disease Progression
Genotype
Heterozygote
Mice
Mice, Inbred C57BL
Mice, Neurologic Mutants
Purkinje Cells / pathology*
Grant Support
ID/Acronym/Agency:
NS20591/NS/NINDS NIH HHS

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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