Linked Life Data

17638298

Source:http://linkedlifedata.com/resource/pubmed/id/17638298

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
13
pubmed:dateCreated
2007-9-20
pubmed:abstractText
Oxidative damage is a known contributor to the pathogenesis of neurodegenerative diseases. Juvenile Batten disease is a progressive neurodegenerative disorder of childhood that results from mutation in Cln3. We have performed an initial characterization of the oxidative burden throughout the CNS in a Cln3(-/-) mouse model for juvenile Batten disease. A survey of multiple regions of the Cln3(-/-) mouse brain revealed a specific reduction of total glutathione, a tripeptide antioxidant molecule, in the cerebellum. Further analysis revealed an increase in protein oxidation not only in the cerebellum but also in the thalamus and primary motor cortex. Additionally, the thalamus was found to have an increase in the amount of a potent antioxidant enzyme, manganese superoxide dismutase (MnSOD), which may be in response to an increase in deleterious superoxide radicals. Colocalization studies indicate that microglia are localized directly adjacent to neurons expressing MnSOD, indicating that microglial activation may be related to the observed oxidative damage. This study helps to provide an initial measure of regions within the CNS of Cln3(-/-) mice that are specifically affected by the loss of CLN3 function and may serve to identify at the neuroanatomical level, the sequence of events that plays a role in the pathogenesis and clinical course of juvenile Batten disease.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0360-4012
pubmed:author
pubmed:copyrightInfo
2007 Wiley-Liss, Inc.
pubmed:issnType
Print
pubmed:volume
85
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
2882-91
pubmed:meshHeading
pubmed-meshheading:17638298-Analysis of Variance, pubmed-meshheading:17638298-Animals, pubmed-meshheading:17638298-Antigens, Differentiation, pubmed-meshheading:17638298-Central Nervous System, pubmed-meshheading:17638298-Disease Models, Animal, pubmed-meshheading:17638298-Gene Expression Regulation, Enzymologic, pubmed-meshheading:17638298-Glutathione, pubmed-meshheading:17638298-Glutathione Peroxidase, pubmed-meshheading:17638298-Membrane Glycoproteins, pubmed-meshheading:17638298-Mice, pubmed-meshheading:17638298-Mice, Knockout, pubmed-meshheading:17638298-Molecular Chaperones, pubmed-meshheading:17638298-Neuronal Ceroid-Lipofuscinoses, pubmed-meshheading:17638298-Oxidation-Reduction, pubmed-meshheading:17638298-Peroxiredoxins, pubmed-meshheading:17638298-Protein Carbonylation, pubmed-meshheading:17638298-Superoxide Dismutase
pubmed:year
2007
pubmed:articleTitle
Progressive oxidative damage in the central nervous system of a murine model for juvenile Batten disease.
pubmed:affiliation
Center for Aging and Developmental Biology, Aab Institute of Biomedical Sciences, University of Rochester School of Medicine and Dentistry, Rochester, New York 14642, USA.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't, Research Support, N.I.H., Extramural