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Expression of X-chr...
Expression of X-chromosome linked inhibitor of apoptosis protein in mature Purkinje cells and in retinal bipolar cells in transgenic mice induces neurodegeneration
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- Korhonen, L. (författare)
- Linköpings universitet,Uppsala universitet,Institutionen för neurovetenskap,Minerva Med Res Inst, Biomedicum Helsinki 2U, FIN-00290 Helsinki, Finland.;Uppsala Univ, Neurobiol Unit, Dept Neurosci, Ctr Biomed, S-75123 Uppsala, Sweden.
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- Hansson, I. (författare)
- Minerva Med Res Inst, Biomedicum Helsinki 2U, FIN-00290 Helsinki, Finland.
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- Maugras, C. (författare)
- CNRS, UMR7102, F-75005 Paris, France.;Univ Paris 06, F-75005 Paris, France.
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- Wehrle, R. (författare)
- CNRS, UMR7102, F-75005 Paris, France.;Univ Paris 06, F-75005 Paris, France.
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- Kairisalo, M. (författare)
- Minerva Med Res Inst, Biomedicum Helsinki 2U, FIN-00290 Helsinki, Finland.
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- Borgkvist, A. (författare)
- Karolinska Institutet
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- Jokitalo, E. (författare)
- Univ Helsinki, Inst Biotechnol, FIN-00014 Helsinki, Finland.
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- Sotelo, C. (författare)
- CNRS, UMR7102, F-75005 Paris, France.;Univ Paris 06, F-75005 Paris, France.;Univ Miguel Hernandez, Inst Neurosci Alicante, Catedra Remedios Caro Almela, Alicante 03550, Spain.
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- Fisone, G. (författare)
- Karolinska Institutet
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- Dusart, I. (författare)
- CNRS, UMR7102, F-75005 Paris, France.;Univ Paris 06, F-75005 Paris, France.
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- Lindholm, D. (författare)
- Uppsala universitet,Institutionen för neurovetenskap,Minerva Med Res Inst, Biomedicum Helsinki 2U, FIN-00290 Helsinki, Finland.;Uppsala Univ, Neurobiol Unit, Dept Neurosci, Ctr Biomed, S-75123 Uppsala, Sweden.
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(creator_code:org_t)
- Elsevier BV, 2008
- 2008
- Engelska.
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Ingår i: Neuroscience. - : Elsevier BV. - 0306-4522 .- 1873-7544. ; 156:3, s. 515-526
- Relaterad länk:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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https://urn.kb.se/re...
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http://kipublication...
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Abstract
Ämnesord
Stäng
- Transgenic mice with overexpression of the caspase-inhibitor, X-chromosome-linked inhibitor of apoptosis protein (XIAP) in Purkinje cell (PC) and in retinal bipolar cells (RBCs) were produced to study the regulation of cell death. Unexpectedly, an increased neurodegeneration was observed in the PCs in these L7-XIAP mice after the third postnatal week with the mice exhibiting severe ataxia. The loss of PCs was independent of Bax as shown by crossing the L7-XIAP mice with Bax gene-deleted mice. Electron microscopy revealed intact organelles in PCs but with the stacking of ER cisterns indicative of cell stress. Immunostaining for cell death proteins showed an increased phosphorylation of c-Jun in the PCs, suggesting an involvement in cell degeneration. Apart from PCs, the number of RBCs was decreased in adult retina in line with the expression pattern for the L7 promoter. The data show that overexpression of the anti-apoptotic protein XIAP in vulnerable neurons leads to enhanced cell death. The mechanisms underlying this neurodegeneration can be related to the effects of XIAP on cell stress and altered cell signaling.
Nyckelord
- cerebellum
- Purkinje cell
- ataxia
- retinal bipolar cells
- XIAP
- Jun N-terminal kinase
- MEDICINE
- MEDICIN
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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Korhonen, L.
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Hansson, I.
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Maugras, C.
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Wehrle, R.
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Kairisalo, M.
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Borgkvist, A.
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visa fler...
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Jokitalo, E.
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Sotelo, C.
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Fisone, G.
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Dusart, I.
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Lindholm, D.
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visa färre...
- Artiklar i publikationen
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Neuroscience
- Av lärosätet
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Uppsala universitet
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Linköpings universitet
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Karolinska Institutet