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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

From: Advancing the pathologic phenotype of giant axonal neuropathy: early involvement of the ocular lens

Fig. 2

Lens epithelial cells in GAN KO mice, age-matched controls and human GAN. a. GAN KO (4-month-old) lens epithelial cells show intracytoplasmic inclusion bodies (H&E original magnification 100X). b. GAN/J KO (24-month-old) lens epithelial cell inclusion bodies (H&E original magnification 100X). c. GAN/J KO (24-month-old) lens epithelial cell inclusion bodies show strong GFAP immunoreactivity (GFAP IHC original magnification 100X). d. Control mouse (24-month-old) histologically unremarkable lens epithelial cells (H&E original magnification 100X). e. Human GAN lens epithelial cells reveal intracytoplasmic inclusion bodies (H&E original magnification 100X, formalin fixed, paraffin embedded tissue. Decedent was a young child with phenotypically typical GAN) (arrows point to one of the numerous intracytoplasmic inclusion bodies). Inset. Lens epithelial cells. Control mouse (24-month-old) (H&E); GAN/J KO (24 -month-old) lens epithelial cell inclusion body (H&E); GAN/J KO (24-month-old) lens epithelial cell inclusion body shows strong GFAP immunoreactivity (GFAP IHC). (N designates nucleus, arrow points to cytoplasmic inclusion body)

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