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Figure 1 | Journal of Neuroinflammation

Figure 1

From: Conditional expression of human β-hexosaminidase in the neurons of Sandhoff disease rescues mice from neurodegeneration but not neuroinflammation

Figure 1

Neuronal β-hexosaminidase (HEX) restitution in Sandhoff mice. (A) The expression of human HEX β/β homodimer (HEXB) open reading frame was targeted to neurons by the Thy1 promoter. (B) Two transgenic mouse lines were generated. (C) β-Hexosaminidase expression was detected by immunohistochemistry employing an antibody raised specifically against human HEXB in histology sections harvested from Thy1-HEXB;HexB−/− brains. (D) As expected, HexB−/− littermates did not express any human HEXB protein. (E) The expression of HEXB (red) was confirmed by (F) double immunofluorescence (yellow overlap) in (G) neurons (green) in the brains of Thy1-HEXB;HexB−/− mice. (H) The lack of HEXB expression was confirmed (I) by double immunofluorescence in (J) MHC-II+ microglia/macrophages. Similarly, there (K) was lack of HEXB expression in astrocytes as detected (L) by the lack of double immunofluorescence in (M) glial fibrillary acidic protein (GFAP)+ astrocytes.

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Journal of Neuroinflammation

ISSN: 1742-2094