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Phenotype Image Detail
Image
Caption Localized glycolipid storage and microgliosis in Hexbtm1Rlp/Hexbtm1Rlp Tg(Hexb-tTA2S,tetO-Hexb)#Tjsa/0 (Hexb-/-HexTg) mice. PAS stained brain sections show regions of the cerebrum such as the primary motor cortex (A) and the striatum (B) are devoid of glycolipid storage that stains magenta. However, storage is a prominent feature in the hindbrain of the same animals. C and D show glycolipid storage in neurons of the brainstem (gigantocellular reticular nucleus) and in the spinal cord grey matter respectively (C, arrowheads; D, dashed line). (E-H) Staining for activated microglia is revealed by brown DAB staining for CD68 and coincides with storage (G, arrowheads show CD68 staining microglia; H, dashed line shows spinal grey matter). (I) PAX2-positive ventral horn interneurons were quantified for Hexbtm1Rlp/Hexbtm1Rlp Tg(Hexb-tTA2S,tetO-Hexb)#Tjsa/0 (Hexb-/-HexTg), Hexbtm1Rlp/Hexbtm1Rlp (Hexb-/-; both humane endpoint) and Hexb+/Hexbtm1Rlp (Hexb+/-; one year old) animals (n = 6, 8 and 6. Bars = mean +/- SEM. *, P<0.05; **, P<0.01; ***P<0.001 - Bonferroni post hoc test). Both Hexbtm1Rlp/Hexbtm1Rlp Tg(Hexb-tTA2S,tetO-Hexb)#Tjsa/0 and Hexbtm1Rlp/Hexbtm1Rlp animals showed loss of PAX2-positive neuron density in multiple regions of the ventral spinal cord compared with Hexb+/Hexbtm1Rlp animals. J and K show PAX2 stained lumbar spinal cord used for quantification. The dashed line encompasses the region quantified. Scale bars: A-C and E-G = 50 um; D, H, J and K = 100 um.
Copyright This image is from Sargeant TJ, PLoS Genet 2012 Sep;8(9):e1002943, and is displayed under the terms of the Creative Commons Attribution 4.0 International License. J:190450
Associated
Alleles
Symbol Name
Hexbtm1Rlp hexosaminidase B; targeted mutation 1, Richard L Proia
Tg(Hexb-tTA2S,tetO-Hexb)#Tjsa transgene insertion, Timothy J Sargeant
Associated
Genotypes
Allelic Composition Genetic Background
Hexbtm1Rlp/Hexbtm1Rlp
Tg(Hexb-tTA2S,tetO-Hexb)#Tjsa/0
involves: 129S4/SvJae * C57BL/6 * CBA

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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
12/10/2024
MGI 6.24
The Jackson Laboratory