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Comment
. 2016 Jul;132(1):145-7.
doi: 10.1007/s00401-016-1581-x. Epub 2016 May 20.

Full ablation of C9orf72 in mice causes immune system-related pathology and neoplastic events but no motor neuron defects

Emma Sudria-Lopez  1 , Max Koppers  1   2 , Marina de Wit  1 , Christiaan van der Meer  1 , Henk-Jan Westeneng  2 , Caroline A C Zundel  1 , Sameh A Youssef  3 , Liesbeth Harkema  3 , Alain de Bruin  3   4 , Jan H Veldink  2 , Leonard H van den Berg  2 , R Jeroen Pasterkamp  5
Affiliations
Comment

Full ablation of C9orf72 in mice causes immune system-related pathology and neoplastic events but no motor neuron defects

Emma Sudria-Lopez et al. Acta Neuropathol. 2016 Jul.
No abstract available

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Figures

Fig. 1
Fig. 1
C9orf72 knockout mice display reduced survival, immune system-related pathology and neoplastic events. a Kaplan–Meier curves show survival rates corrected for gender and body weight. C9orf72 knockout mice show reduced survival as compared to littermate controls (Hazard ratio = 19.0; 95 %, CI: 2.4–150.2, P = 0.005). Wild-type controls n = 24; C9orf72 knockout n = 29. b Gross image showing enlarged lymph nodes (LNs; black arrows) and splenomegaly (white arrow) in a C9orf72 knockout mouse (12 months of age). c–e B-cell lymphoma in the submandibular LNs of C9orf72 knockout mouse. Nodal tissue is effaced by a monotypic cell population composed of B220/CD45R-positive lymphocytes (B cells). Note the high proliferation rate of the neoplastic lymphocytes as indicated by immunostaining for Ki67 (proliferation marker). f–h Histiocytic sarcoma in the liver of C9orf72 knockout mouse. Hepatic sinusoids are filled with atypical histiocytes and multinucleated giant cells that stain positive for the macrophage lineage marker F4/80 and exhibit a high proliferation rate, as evidenced by Ki67 immunostaining. i–k Histiocytic sarcoma in lung vasculature in C9orf72 knockout mouse. Pulmonary blood vessels are filled with atypical and multinucleated giant cells that immunostain for F4/80 and Ki67. H&E hematoxylin and eosin. Scale bar 1.3 cm (b), 65 μm (c), 40 μm (d–h), 125 μm (i), and 90 μm (j, k)
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References

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