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Histochemical and immunohistochemical staining of cornea and retina in unaffected and MPS I affected dogs. Alcian blue (A–B), luxol fast blue (C–D), and lysosomal integral membrane protein 2 <t>(LIMP2)</t> immunohistochemistry (E–F), 400x. (A) Alcian blue (AB) stained cornea from a 1-year-old unaffected control dog. B) AB stained cornea from a 1-year-old MPS I affected dog. Markedly vacuolated stromal cells contain AB positive storage material (arrow). (C) Luxol fast blue (LFB) stained retina from a 2.1-year-old unaffected control dog. (D) LFB stained retina from a 1-year-old MPS I affected dog. Rare retinal ganglion cells contain LFB positive storage material (arrow). (E) LIMP2 immunoreactivity in the retina of a 2.1-year-old unaffected control. (F) Retinal LIMP2 immunoreactivity in a 2.1-year-old MPS I affected dog. There is increased immunoreactivity in MPS I affected retinas, predominantly in the retinal ganglion cell layer (arrow)
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Histochemical and immunohistochemical staining of cornea and retina in unaffected and MPS I affected dogs. Alcian blue (A–B), luxol fast blue (C–D), and lysosomal integral membrane protein 2 (LIMP2) immunohistochemistry (E–F), 400x. (A) Alcian blue (AB) stained cornea from a 1-year-old unaffected control dog. B) AB stained cornea from a 1-year-old MPS I affected dog. Markedly vacuolated stromal cells contain AB positive storage material (arrow). (C) Luxol fast blue (LFB) stained retina from a 2.1-year-old unaffected control dog. (D) LFB stained retina from a 1-year-old MPS I affected dog. Rare retinal ganglion cells contain LFB positive storage material (arrow). (E) LIMP2 immunoreactivity in the retina of a 2.1-year-old unaffected control. (F) Retinal LIMP2 immunoreactivity in a 2.1-year-old MPS I affected dog. There is increased immunoreactivity in MPS I affected retinas, predominantly in the retinal ganglion cell layer (arrow)

Journal: Journal of inherited metabolic disease

Article Title: Clinical and pathological characterization of ophthalmic disease in a canine model of mucopolysaccharidosis type I

doi: 10.1002/jimd.12587

Figure Lengend Snippet: Histochemical and immunohistochemical staining of cornea and retina in unaffected and MPS I affected dogs. Alcian blue (A–B), luxol fast blue (C–D), and lysosomal integral membrane protein 2 (LIMP2) immunohistochemistry (E–F), 400x. (A) Alcian blue (AB) stained cornea from a 1-year-old unaffected control dog. B) AB stained cornea from a 1-year-old MPS I affected dog. Markedly vacuolated stromal cells contain AB positive storage material (arrow). (C) Luxol fast blue (LFB) stained retina from a 2.1-year-old unaffected control dog. (D) LFB stained retina from a 1-year-old MPS I affected dog. Rare retinal ganglion cells contain LFB positive storage material (arrow). (E) LIMP2 immunoreactivity in the retina of a 2.1-year-old unaffected control. (F) Retinal LIMP2 immunoreactivity in a 2.1-year-old MPS I affected dog. There is increased immunoreactivity in MPS I affected retinas, predominantly in the retinal ganglion cell layer (arrow)

Article Snippet: Sections were then incubated overnight at 4°C with rabbit anti-LIMP2 (PA3–16802, Invitrogen; 1:500).

Techniques: Immunohistochemical staining, Staining, Membrane, Immunohistochemistry, Control

Mean retinal LIMP2 immunoreactivity in 6 unaffected and 6 MPS I affected dogs of clinical age. (A) Mean LIMP2 immunolabeling is significantly increased ( p -value 0.0094) in MPS I dogs compared to unaffected controls. Error bars represent standard error of the mean. (B) Individual animal data. LIMP2 measurements were taken from four regions of the retina. Each bar represents the mean and standard deviation. At all timepoints, LIMP2 immunoreactivity is increased compared to similarly aged controls but does not appear to increase with age. X-axis is age in years

Journal: Journal of inherited metabolic disease

Article Title: Clinical and pathological characterization of ophthalmic disease in a canine model of mucopolysaccharidosis type I

doi: 10.1002/jimd.12587

Figure Lengend Snippet: Mean retinal LIMP2 immunoreactivity in 6 unaffected and 6 MPS I affected dogs of clinical age. (A) Mean LIMP2 immunolabeling is significantly increased ( p -value 0.0094) in MPS I dogs compared to unaffected controls. Error bars represent standard error of the mean. (B) Individual animal data. LIMP2 measurements were taken from four regions of the retina. Each bar represents the mean and standard deviation. At all timepoints, LIMP2 immunoreactivity is increased compared to similarly aged controls but does not appear to increase with age. X-axis is age in years

Article Snippet: Sections were then incubated overnight at 4°C with rabbit anti-LIMP2 (PA3–16802, Invitrogen; 1:500).

Techniques: Immunolabeling, Standard Deviation