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Caption Neurodegenerative changes in Purkinje cell-specific knockout of Xpa in Ercc6tm1Gvh/Ercc6tm1Gvh (Csb-/-) mice. F-J) Photomicrographs of sagittal cerebellar sections of 25-week old Ercc6tm1Gvh/Ercc6tm1Gvh (Csb-/-) (F1-J1) and Ercc6tm1Gvh/Ercc6tm1Gvh Xpatm1Gvh/Xpatm1Hvs Tg(Pcp2-cre)2Mpin/0 (Csb-/- Xpac/- L7-Cre) mice (F2-J2). F) Silver degeneration staining shows a high level of argyrophilic fibers in the deep cerebellar nuclei (DCN) and the white matter (wm) of Ercc6tm1Gvh/Ercc6tm1Gvh Xpatm1Gvh/Xpatm1Hvs Tg(Pcp2-cre)2Mpin/0 cerebellum (arrow in F2). G) GFAP-immunoperoxidase staining shows increased GFAP staining in the molecular (ml) and Purkinje cell (Pcl) layers of Ercc6tm1Gvh/Ercc6tm1Gvh Xpatm1Gvh/Xpatm1Hvs Tg(Pcp2-cre)2Mpin/0 cerebellar cortex, while no changes occur in the granule cell layer (Gcl). H) Calbindin immunoperoxidase staining reveals calbindin-negative regions in the molecular layer (arrow in H2) of Ercc6tm1Gvh/Ercc6tm1Gvh Xpatm1Gvh/Xpatm1Hvs Tg(Pcp2-cre)2Mpin/0 cerebellar cortex. I) A number of Purkinje cells showed strong nuclear ATF3 staining (arrow in I2). Note that the ATF3 antibody also outlines Purkinje cells because of non-specific cytoplasmatic staining of these cells. J) Active caspase 3 antibody stains sporadic Purkinje cells in Ercc6tm1Gvh/Ercc6tm1Gvh Xpatm1Gvh/Xpatm1Hvs Tg(Pcp2-cre)2Mpin/0 cerebellar cortex (arrow head in J2). Calibration Bars: 500 um (F2); 50 um (H2), 25 um (J2).
Copyright This image is from Jaarsma D, PLoS Genet 2011 Dec;7(12):e1002405, an open-access article distributed under the terms of the Creative Commons Attribution License. J:179808
Associated
Alleles
Symbol Name
Ercc6tm1Gvh excision repair cross-complementing rodent repair deficiency, complementation group 6; targeted mutation 1, Gijsbertus van der Horst
Tg(Pcp2-cre)2Mpin transgene insertion 2, Max-Planck-Institute of Neurobiology
Xpatm1Gvh xeroderma pigmentosum, complementation group A; targeted mutation 1, Gijsbertus T J van der Horst
Xpatm1Hvs xeroderma pigmentosum, complementation group A; targeted mutation 1, Harry van Steeg
Associated
Genotypes
Allelic Composition Genetic Background
Ercc6tm1Gvh/Ercc6tm1Gvh
Xpatm1Gvh/Xpatm1Hvs
Tg(Pcp2-cre)2Mpin/0
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/08/2014
MGI 5.17
The Jackson Laboratory