Source:http://linkedlifedata.com/resource/pubmed/id/17656100
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
1
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| pubmed:dateCreated |
2007-9-25
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| pubmed:abstractText |
Infantile neuronal ceroid lipofuscinosis (INCL) is a severe neurodegenerative disorder of children, characterized by selective death of neocortical neurons. To understand early disease mechanisms in INCL, we have studied Ppt1(Deltaex4) knock-out mouse neurons in culture and acute brain slices. Global transcript profiling showed deregulation of key neuronal functions in knock-out mice including cholesterol metabolism, neuronal maturation, and calcium homeostasis. Cholesterol metabolism showed major changes; sterol biosynthesis was enhanced and steady-state amounts of sterols were altered at the cellular level. Changes were also present in early maturation of Ppt1(Deltaex4) neurons indicated by increased proliferative capacity of neuronal stem cells. Knock-out neurons presented unaltered electrophysiological properties suggesting uncompromised synaptic function in young animals. However, knock-out neurons exhibited more efficient recovery from glutamate-induced calcium transients, possibly indicating neuroprotective activation. This study established that the neuronal deregulation in INCL is linked to neuronal maturation, lipid metabolism and calcium homeostasis.
|
| pubmed:language |
eng
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| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Oct
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| pubmed:issn |
0969-9961
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| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:volume |
28
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| pubmed:owner |
NLM
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| pubmed:authorsComplete |
Y
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| pubmed:pagination |
52-64
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| pubmed:meshHeading |
pubmed-meshheading:17656100-Animals,
pubmed-meshheading:17656100-Calcium,
pubmed-meshheading:17656100-Cell Differentiation,
pubmed-meshheading:17656100-Cell Proliferation,
pubmed-meshheading:17656100-Cells, Cultured,
pubmed-meshheading:17656100-Cholesterol,
pubmed-meshheading:17656100-Fluorescent Antibody Technique,
pubmed-meshheading:17656100-Gene Expression Profiling,
pubmed-meshheading:17656100-Hippocampus,
pubmed-meshheading:17656100-Homeostasis,
pubmed-meshheading:17656100-Mice,
pubmed-meshheading:17656100-Mice, Congenic,
pubmed-meshheading:17656100-Mice, Inbred C57BL,
pubmed-meshheading:17656100-Mice, Knockout,
pubmed-meshheading:17656100-Neuronal Ceroid-Lipofuscinoses,
pubmed-meshheading:17656100-Neurons,
pubmed-meshheading:17656100-Organ Culture Techniques,
pubmed-meshheading:17656100-Patch-Clamp Techniques,
pubmed-meshheading:17656100-Stem Cells,
pubmed-meshheading:17656100-Synapses,
pubmed-meshheading:17656100-Thiolester Hydrolases
|
| pubmed:year |
2007
|
| pubmed:articleTitle |
Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction.
|
| pubmed:affiliation |
National Public Health Institute, Department of Molecular Medicine, Biomedicum Helsinki, PO Box 104, 00251 Helsinki, Finland.
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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