Phenotype Image Detail

Image

Caption

Neurodegenerative changes in Purkinje cell-specific knockout of Xpa in Ercc6^tm1Gvh/Ercc6^tm1Gvh (Csb^-/-) mice. F-J) Photomicrographs of sagittal cerebellar sections of 25-week old Ercc6^tm1Gvh/Ercc6^tm1Gvh (Csb^-/-) (F1-J1) and Ercc6^tm1Gvh/Ercc6^tm1Gvh Xpa^tm1Gvh/Xpa^tm1Hvs Tg(Pcp2-cre)2Mpin/0 (Csb^-/- Xpa^c/- 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 Ercc6^tm1Gvh/Ercc6^tm1Gvh Xpa^tm1Gvh/Xpa^tm1Hvs 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 Ercc6^tm1Gvh/Ercc6^tm1Gvh Xpa^tm1Gvh/Xpa^tm1Hvs 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 Ercc6^tm1Gvh/Ercc6^tm1Gvh Xpa^tm1Gvh/Xpa^tm1Hvs 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 Ercc6^tm1Gvh/Ercc6^tm1Gvh Xpa^tm1Gvh/Xpa^tm1Hvs Tg(Pcp2-cre)2Mpin/0 cerebellar cortex (arrow head in J2). Calibration Bars: 500 um (F2); 50 um (H2), 25 um (J2).

This image is from Jaarsma D, PLoS Genet 2011 Dec;7(12):e1002405, and is displayed under the terms of the Creative Commons Attribution 4.0 International License. J:179808

Associated
Alleles

Symbol	Name
Ercc6^tm1Gvh	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
Xpa^tm1Gvh	xeroderma pigmentosum, complementation group A; targeted mutation 1, Gijsbertus T J van der Horst
Xpa^tm1Hvs	xeroderma pigmentosum, complementation group A; targeted mutation 1, Harry van Steeg

Associated
Genotypes

Allelic Composition	Genetic Background
Ercc6^tm1Gvh/Ercc6^tm1Gvh Xpa^tm1Gvh/Xpa^tm1Hvs Tg(Pcp2-cre)2Mpin/0	involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6J

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