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| Caption | Neuropathology of Arsgtm1Tdi/Arsgtm1Tdi (Arsg KO) mice. (A and B) Toluidine blue-stained Epon-embedded sections of the CNS reveal storage vacuoles (arrows) in Purkinje cells (A) and perivascular macrophages (B). (C) Storage vacuoles in both Purkinje cells (long arrow) and macrophages (short arrows) show positive periodic acid-Schiff (PAS) staining. (D) TEM reveals heterogeneous, partially water-soluble, and finely granular to lamellar storage material with variable electron density in Purkinje cells. (E and E') Fluorescence microscopy of unstained sections shows autofluorescence in the cerebellar cortex of mutant mice but not in WT animals. (F and F') Autofluorescent storage material (red) colocalizes with ring-like structures of the lysosomal membrane protein Lamp1 (green), indicating its lysosomal origin. (G and G') Considerable loss of Purkinje cells can be observed by 12 months of age in mutant mice as assessed by Calbindin immunohistochemistry. (Inset) Remaining Purkinje cells show swollen dendrites (arrows) and somata (arrowhead). (H-J') Profound activation of microglia transforming to an amoeboid to phagocytic morphology (H and H', I and I') and hypertrophy of astrocytes (J and J') occur in mutant animals in the cerebellar cortex as assessed with markers for microglia/macrophages (CD68) and astrocytes (GFAP) (age 12 months). (K and L) Mutant mice showed a progressive exploratory deficit in the open-field test as indicated by reduced distance traveled (K) and time spent (L) in the center. (M) Lower step-through latencies indicate impaired passive-avoidance learning in mutant mice. (N) Water-maze probe trial shows impaired visuo-spatial memory for the platform location in mutant mice. For K-N, n = 20, age 12 months; error bars indicate SEM. | ||||
| Copyright | This image is from Kowalewski B, Proc Natl Acad Sci U S A 2012 Jun 26;109(26):10310-5. Copyright 2012 National Academy of Sciences, U.S.A. J:185592 | ||||
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Associated Alleles |
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Associated Genotypes |
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/12/2024 MGI 6.24 |
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