Document Detail


Canine hereditary ceroid lipofuscinosis.
MedLine Citation:
PMID:  7117302     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Dogs with an inherited form of ceroid lipofuscinosis are ataxic, blind and demented. During the disease process, they undergo severe cerebrocerebellar atrophy with storage of autofluorescent, lipid peroxide-positive reacting substances whose ultrastructure resembles 'fingerprint' patterns of membranous lamellae. The retina and RPE also undergo pathologic changes. Most important is the inverse relationship between loss of RPE melanin and increased deposition of ceroid. These pathological events in brain and eye lead to altered EEG, ERG and VEP activity. This inbred strain of English setters fulfills essentially all the criteria as a model for the human disease and will prove useful in the future for therapeutic trials.
Authors:
D Armstrong; N Koppang; S E Nilsson
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  European neurology     Volume:  21     ISSN:  0014-3022     ISO Abbreviation:  Eur. Neurol.     Publication Date:  1982  
Date Detail:
Created Date:  1982-12-02     Completed Date:  1982-12-02     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0150760     Medline TA:  Eur Neurol     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  147-56     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Animals
Atrophy
Blindness / pathology,  veterinary
Brain Diseases / genetics,  pathology,  veterinary*
Cerebellar Ataxia / genetics,  veterinary
Ceroid / metabolism*
Dog Diseases / genetics*,  pathology
Dogs
Evoked Potentials, Visual
Lipofuscin / metabolism*
Microscopy, Electron
Photoreceptor Cells / pathology
Pigments, Biological / metabolism*
Grant Support
ID/Acronym/Agency:
EY 02627/EY/NEI NIH HHS; KO4 EY 00130/EY/NEI NIH HHS
Chemical
Reg. No./Substance:
0/Ceroid; 0/Lipofuscin; 0/Pigments, Biological

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


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