L773:1471 2202
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| Fältnamn | Indikatorer | Metadata |
|---|---|---|
| 000 | 03420naa a2200361 4500 | |
| 001 | oai:lup.lub.lu.se:da50b3d4-eca2-4d78-aeb3-6d73bb65d049 | |
| 003 | SwePub | |
| 008 | 160401s2005 | |||||||||||000 ||eng| | |
| 024 | 7 | a https://lup.lub.lu.se/record/2445392 URI |
| 024 | 7 | a https://doi.org/10.1186/1471-2202-6-272 DOI |
| 040 | a (SwePub)lu | |
| 041 | a engb eng | |
| 042 | 9 SwePub | |
| 072 | 7 | a art2 swepub-publicationtype |
| 072 | 7 | a ref2 swepub-contenttype |
| 100 | 1 | a Lonka, L4 aut |
| 245 | 1 0 | a The neuronal ceroid lipofuscinosis Cln8 gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy |
| 264 | 1 | b Springer Science and Business Media LLC,c 2005 |
| 520 | a Background: The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative disorders characterized by accumulation of autofluorescent material in many tissues, especially in neurons. Mutations in the CLN8 gene, encoding an endoplasmic reticulum ( ER) transmembrane protein of unknown function, underlie NCL phenotypes in humans and mice. The human phenotype is characterized by epilepsy, progressive psychomotor deterioration and visual loss, while motor neuron degeneration (mnd) mice with a Cln8 mutation show progressive motor neuron dysfunction and retinal degeneration. Results: We investigated spatial and temporal expression of Cln8 messenger ribonucleic acid ( mRNA) using in situ hybridization, reverse transcriptase polymerase chain reaction (RT-PCR) and northern blotting. Cln8 is ubiquitously expressed at low levels in embryonic and adult tissues. In prenatal embryos Cln8 is most prominently expressed in the developing gastrointestinal tract, dorsal root ganglia (DRG) and brain. In postnatal brain the highest expression is in the cortex and hippocampus. Expression of Cln8 mRNA in the central nervous system (CNS) was also analyzed in the hippocampal electrical kindling model of epilepsy, in which Cln8 expression was rapidly upregulated in hippocampal pyramidal and granular neurons. Conclusion: Expression of Cln8 in the developing and mature brain suggests roles for Cln8 in maturation, differentiation and supporting the survival of different neuronal populations. The relevance of Cln8 up-regulation in hippocampal neurons of kindled mice should be further explored. | |
| 650 | 7 | a MEDICIN OCH HÄLSOVETENSKAPx Medicinska och farmaceutiska grundvetenskaperx Neurovetenskaper0 (SwePub)301052 hsv//swe |
| 650 | 7 | a MEDICAL AND HEALTH SCIENCESx Basic Medicinex Neurosciences0 (SwePub)301052 hsv//eng |
| 700 | 1 | a Aalto, A4 aut |
| 700 | 1 | a Kopra, O4 aut |
| 700 | 1 | a Kuronen, M4 aut |
| 700 | 1 | a Kokaia, Zaalu Lund University,Lunds universitet,Neurologi, Lund,Sektion IV,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Neurology, Lund,Section IV,Department of Clinical Sciences, Lund,Faculty of Medicine4 aut0 (Swepub:lu)neur-zko |
| 700 | 1 | a Saarma, M4 aut |
| 700 | 1 | a Lehesjoki, A E4 aut |
| 710 | 2 | a Neurologi, Lundb Sektion IV4 org |
| 773 | 0 | t BMC Neuroscienced : Springer Science and Business Media LLCg 6:27q 6:27x 1471-2202 |
| 856 | 4 | u http://dx.doi.org/10.1186/1471-2202-6-27x freey FULLTEXT |
| 856 | 4 | u https://bmcneurosci.biomedcentral.com/track/pdf/10.1186/1471-2202-6-27 |
| 856 | 4 8 | u https://lup.lub.lu.se/record/244539 |
| 856 | 4 8 | u https://doi.org/10.1186/1471-2202-6-27 |
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