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    
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.
N Hadj-Sahraoui; F Frederic; H Zanjani; N Delhaye-Bouchaud; K Herrup; J Mariani
Related Documents :
8176414 - The fate of schwann cells in peripheral nerve allografts.
15671664 - Olfactory ensheathing cells and repair of brain and spinal cord injuries.
21573494 - Interstitial cells of cajal (icc)-like-c-kit positive cells are involved in gastritis a...
20943904 - Intrinsic plasticity complements long-term potentiation in parallel fiber input gain co...
1000594 - The occurrence of argyrophilic and argentaffin cells in the gut of ciona intestinalis l.
7249064 - Supraependymal cell clusters in the rat brain.
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    
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.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Aging / genetics,  pathology*
Atrophy / genetics,  pathology
Cell Death / genetics
Cerebellar Diseases / genetics,  pathology*
Cerebellum / pathology*
Dendrites / pathology*
Disease Progression
Mice, Inbred C57BL
Mice, Neurologic Mutants
Purkinje Cells / pathology*
Grant Support

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

Previous Document:  Immunolocalization of the calcium binding S100A1, S100A5 and S100A6 proteins in the dog cochlea duri...
Next Document:  Developmental changes of (3)H-labelled mu-opioid receptors in brainstems of intra-uterine growth-res...