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rabbit antihuman α gal a primary antibody  (Bio-Rad)


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    Bio-Rad rabbit antihuman α gal a primary antibody
    hR301Q α-Gal A Tg/KO mice have reduced α-Gal A and elevated GL-3 levels. (a) α-Gal A and GL-3 levels were assessed in tissue lysates prepared from skin, heart, and kidney of 12-week-old male GLA KO (open bars), hR301Q α-Gal A Tg/KO (gray bars), and wild-type C57BL/6 (black bars) mice. α-Gal A activity in hR301Q α-Gal A Tg/KO and GLA KO mice was significantly lower than that of wild-type mice (*P < 0.05, t-test); α-Gal A activity in hR301Q α-Gal A Tg/KO mice was significantly higher than that of GLA KO mice (#P < 0.05, t-test). GL-3 levels in hR301Q α-Gal A Tg/KO and GLA KO mice were significantly higher than those of wild-type mice (*P < 0.05, t-test). Each bar represents the mean ± SEM from four mice per group analyzed in triplicate. (b) Cell-type specific GL-3 staining was assessed by immunohistochemistry in GLA KO, hR301Q α-Gal A Tg/KO, and wild-type C57BL/6 mouse tissues. GL-3 staining is represented as brown spots (black arrows); nuclei are represented as green spots (stained with methyl green). The data shown are representative photomicrographs from four 12-week-old male mice using ×20 magnification, except for the glomeruli, which are representative of four 7-month-old male mice using ×40 magnification. α-Gal A, α-galactosidase A; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Rabbit Antihuman α Gal A Primary Antibody, supplied by Bio-Rad, used in various techniques. Bioz Stars score: 96/100, based on 1736 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/rabbit antihuman α gal a primary antibody/product/Bio-Rad
    Average 96 stars, based on 1736 article reviews
    rabbit antihuman α gal a primary antibody - by Bioz Stars, 2026-03
    96/100 stars

    Images

    1) Product Images from "The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease"

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    Journal:

    doi: 10.1038/mt.2009.220

    hR301Q α-Gal A Tg/KO mice have reduced α-Gal A and elevated GL-3 levels. (a) α-Gal A and GL-3 levels were assessed in tissue lysates prepared from skin, heart, and kidney of 12-week-old male GLA KO (open bars), hR301Q α-Gal A Tg/KO (gray bars), and wild-type C57BL/6 (black bars) mice. α-Gal A activity in hR301Q α-Gal A Tg/KO and GLA KO mice was significantly lower than that of wild-type mice (*P < 0.05, t-test); α-Gal A activity in hR301Q α-Gal A Tg/KO mice was significantly higher than that of GLA KO mice (#P < 0.05, t-test). GL-3 levels in hR301Q α-Gal A Tg/KO and GLA KO mice were significantly higher than those of wild-type mice (*P < 0.05, t-test). Each bar represents the mean ± SEM from four mice per group analyzed in triplicate. (b) Cell-type specific GL-3 staining was assessed by immunohistochemistry in GLA KO, hR301Q α-Gal A Tg/KO, and wild-type C57BL/6 mouse tissues. GL-3 staining is represented as brown spots (black arrows); nuclei are represented as green spots (stained with methyl green). The data shown are representative photomicrographs from four 12-week-old male mice using ×20 magnification, except for the glomeruli, which are representative of four 7-month-old male mice using ×40 magnification. α-Gal A, α-galactosidase A; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Figure Legend Snippet: hR301Q α-Gal A Tg/KO mice have reduced α-Gal A and elevated GL-3 levels. (a) α-Gal A and GL-3 levels were assessed in tissue lysates prepared from skin, heart, and kidney of 12-week-old male GLA KO (open bars), hR301Q α-Gal A Tg/KO (gray bars), and wild-type C57BL/6 (black bars) mice. α-Gal A activity in hR301Q α-Gal A Tg/KO and GLA KO mice was significantly lower than that of wild-type mice (*P < 0.05, t-test); α-Gal A activity in hR301Q α-Gal A Tg/KO mice was significantly higher than that of GLA KO mice (#P < 0.05, t-test). GL-3 levels in hR301Q α-Gal A Tg/KO and GLA KO mice were significantly higher than those of wild-type mice (*P < 0.05, t-test). Each bar represents the mean ± SEM from four mice per group analyzed in triplicate. (b) Cell-type specific GL-3 staining was assessed by immunohistochemistry in GLA KO, hR301Q α-Gal A Tg/KO, and wild-type C57BL/6 mouse tissues. GL-3 staining is represented as brown spots (black arrows); nuclei are represented as green spots (stained with methyl green). The data shown are representative photomicrographs from four 12-week-old male mice using ×20 magnification, except for the glomeruli, which are representative of four 7-month-old male mice using ×40 magnification. α-Gal A, α-galactosidase A; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Techniques Used: Activity Assay, Staining, Immunohistochemistry, Knock-Out, Transgenic Assay

    DGJ administration increases α-Gal A activity and reduces GL-3 in hR301Q α-Gal A Tg/KO mice. Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 4 weeks at the indicated doses. Lysates from skin, heart, and kidney were subsequently tested for (a) α-Gal A activity and (b,c) GL-3 levels. (a) Significant increases in α-Gal A activity were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent increase in α-Gal A activity (post hoc analysis, P < 0.05). Insets: Tissue α-Gal A protein levels were determined by western blotting. A dose-dependent increase in the 46 kd mature form of α-Gal A was seen in all tissues. Each lane on the blot contains tissue lysate from a single animal. The western blot shown is representative of two experiments. (b) Significant reductions in GL-3 levels were seen in all three tissues as measured by LC-MS/MS (*P < 0.05 versus untreated, t-test). Blue bars (a,b) represent α-Gal A and GL-3 levels from age-matched untreated wild-type C57BL/6 mice. Each bar represents pooled data from three independent studies with the mean ± SEM of 21 hR301Q α-Gal A Tg/KO mice/group or four untreated wild-type mice, all analyzed in triplicate. (c) Cell type–specific reduction of GL-3 in hR301Q α-Gal A Tg/KO mice administered DGJ (100 mg/kg per day) ad libitum in drinking water was assessed by immunohistochemistry. GL-3 staining is represented as brown spots denoted with black arrows. The data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, insets: ×40). α-Gal A, α-galactosidase A; DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Figure Legend Snippet: DGJ administration increases α-Gal A activity and reduces GL-3 in hR301Q α-Gal A Tg/KO mice. Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 4 weeks at the indicated doses. Lysates from skin, heart, and kidney were subsequently tested for (a) α-Gal A activity and (b,c) GL-3 levels. (a) Significant increases in α-Gal A activity were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent increase in α-Gal A activity (post hoc analysis, P < 0.05). Insets: Tissue α-Gal A protein levels were determined by western blotting. A dose-dependent increase in the 46 kd mature form of α-Gal A was seen in all tissues. Each lane on the blot contains tissue lysate from a single animal. The western blot shown is representative of two experiments. (b) Significant reductions in GL-3 levels were seen in all three tissues as measured by LC-MS/MS (*P < 0.05 versus untreated, t-test). Blue bars (a,b) represent α-Gal A and GL-3 levels from age-matched untreated wild-type C57BL/6 mice. Each bar represents pooled data from three independent studies with the mean ± SEM of 21 hR301Q α-Gal A Tg/KO mice/group or four untreated wild-type mice, all analyzed in triplicate. (c) Cell type–specific reduction of GL-3 in hR301Q α-Gal A Tg/KO mice administered DGJ (100 mg/kg per day) ad libitum in drinking water was assessed by immunohistochemistry. GL-3 staining is represented as brown spots denoted with black arrows. The data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, insets: ×40). α-Gal A, α-galactosidase A; DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Techniques Used: Activity Assay, Western Blot, Liquid Chromatography with Mass Spectroscopy, Immunohistochemistry, Staining, Knock-Out, Transgenic Assay

    Long-term (24-week) administration of DGJ results in greater GL-3 reduction compared to 4-week administration. (a) Four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks at the indicated doses. GL-3 levels were subsequently measured in skin, heart, and kidney lysates by LC-MS/MS. Significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent decrease in GL-3 levels (post hoc analysis, P < 0.05). Each bar represents the mean ± SEM of 7–8 mice/dose group analyzed in triplicate. (b) Cell type–specific GL-3 reductions were assessed by immunohistochemistry. GL-3 staining is represented as brown spots. Data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, except glomeruli: ×40). DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Figure Legend Snippet: Long-term (24-week) administration of DGJ results in greater GL-3 reduction compared to 4-week administration. (a) Four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks at the indicated doses. GL-3 levels were subsequently measured in skin, heart, and kidney lysates by LC-MS/MS. Significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent decrease in GL-3 levels (post hoc analysis, P < 0.05). Each bar represents the mean ± SEM of 7–8 mice/dose group analyzed in triplicate. (b) Cell type–specific GL-3 reductions were assessed by immunohistochemistry. GL-3 staining is represented as brown spots. Data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, except glomeruli: ×40). DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Techniques Used: Liquid Chromatography with Mass Spectroscopy, Immunohistochemistry, Staining, Knock-Out, Transgenic Assay

    Effect of 4- and 24-week DGJ administration on tissue GL-3 levels in hR301Q α-Gal A Tg/KO mice
    Figure Legend Snippet: Effect of 4- and 24-week DGJ administration on tissue GL-3 levels in hR301Q α-Gal A Tg/KO mice

    Techniques Used:

    Less frequent DGJ administration results in greater GL-3 reduction. (a) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered drinking water (red dotted lines) or DGJ [100 mg/kg per day (red solid lines) ad libitum in drinking water] for 4 weeks, followed by a washout period (drinking water only) of up to 7 days. Groups of mice were then euthanized on days 0, 1, 3, 5, or 7 after DGJ withdrawal. Skin, heart, and kidney were collected at each time point, and α-Gal A activity and DGJ levels (blue lines) were measured in tissue lysates. Significantly increased α-Gal A activity was sustained in all three tissues for up to 7 days (*P < 0.05 versus untreated, t-test). For the α-Gal A activity, each data point represents the mean ± SEM of 7–8 mice/time point analyzed in triplicate; data were normalized to untreated levels. For tissue DGJ levels, each data point represents the mean ± SEM for five mice/time point. The limit of quantitation for DGJ in skin, heart, and kidney was 6, 4, and 10 ng/g, respectively. (b) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ (300 mg/kg per day) ad libitum in drinking water for 4 weeks either daily or less frequently using four cycles of a “4 on/3 off” regimen. GL-3 levels were subsequently measured in lysates from skin, heart, and kidney by LC-MS/MS. Significant reductions in GL-3 levels were seen in all tissues (*P < 0.05 versus untreated, t-test). GL-3 levels in skin, heart, and kidney also showed significantly greater reductions with less frequent compared to daily administration (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents pooled data from three independent studies with the mean ± SEM of 21 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Figure Legend Snippet: Less frequent DGJ administration results in greater GL-3 reduction. (a) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered drinking water (red dotted lines) or DGJ [100 mg/kg per day (red solid lines) ad libitum in drinking water] for 4 weeks, followed by a washout period (drinking water only) of up to 7 days. Groups of mice were then euthanized on days 0, 1, 3, 5, or 7 after DGJ withdrawal. Skin, heart, and kidney were collected at each time point, and α-Gal A activity and DGJ levels (blue lines) were measured in tissue lysates. Significantly increased α-Gal A activity was sustained in all three tissues for up to 7 days (*P < 0.05 versus untreated, t-test). For the α-Gal A activity, each data point represents the mean ± SEM of 7–8 mice/time point analyzed in triplicate; data were normalized to untreated levels. For tissue DGJ levels, each data point represents the mean ± SEM for five mice/time point. The limit of quantitation for DGJ in skin, heart, and kidney was 6, 4, and 10 ng/g, respectively. (b) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ (300 mg/kg per day) ad libitum in drinking water for 4 weeks either daily or less frequently using four cycles of a “4 on/3 off” regimen. GL-3 levels were subsequently measured in lysates from skin, heart, and kidney by LC-MS/MS. Significant reductions in GL-3 levels were seen in all tissues (*P < 0.05 versus untreated, t-test). GL-3 levels in skin, heart, and kidney also showed significantly greater reductions with less frequent compared to daily administration (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents pooled data from three independent studies with the mean ± SEM of 21 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Techniques Used: Activity Assay, Quantitation Assay, Liquid Chromatography with Mass Spectroscopy, Knock-Out, Transgenic Assay

    Long-term DGJ administration reduces accumulated tissue GL-3 in aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. GL-3 levels were measured in lysates of skin, heart, and kidney by LC-MS/MS. Dose-dependent and significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). Reduction of GL-3 in skin and heart was significantly greater after less frequent compared to daily administration of 300 mg/kg DGJ (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Figure Legend Snippet: Long-term DGJ administration reduces accumulated tissue GL-3 in aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. GL-3 levels were measured in lysates of skin, heart, and kidney by LC-MS/MS. Dose-dependent and significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). Reduction of GL-3 in skin and heart was significantly greater after less frequent compared to daily administration of 300 mg/kg DGJ (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Techniques Used: Liquid Chromatography with Mass Spectroscopy, Knock-Out, Transgenic Assay

    Effect of DGJ on plasma GL-3 levels in hR301Q α-Gal A Tg/KO mice
    Figure Legend Snippet: Effect of DGJ on plasma GL-3 levels in hR301Q α-Gal A Tg/KO mice

    Techniques Used:

    DGJ increases α-Gal A and decreases GL-3 levels in brain of aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 12 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. Brain α-Gal A and GL-3 levels were subsequently measured. Significant increases in α-Gal A and concomitant reductions in GL-3 were seen with DGJ administration (*P < 0.05 versus untreated, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Figure Legend Snippet: DGJ increases α-Gal A and decreases GL-3 levels in brain of aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 12 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. Brain α-Gal A and GL-3 levels were subsequently measured. Significant increases in α-Gal A and concomitant reductions in GL-3 were seen with DGJ administration (*P < 0.05 versus untreated, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Techniques Used: Knock-Out, Transgenic Assay



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    rabbit antihuman α gal a primary antibody  (Bio-Rad)
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    Bio-Rad rabbit antihuman α gal a primary antibody
    hR301Q α-Gal A Tg/KO mice have reduced α-Gal A and elevated GL-3 levels. (a) α-Gal A and GL-3 levels were assessed in tissue lysates prepared from skin, heart, and kidney of 12-week-old male GLA KO (open bars), hR301Q α-Gal A Tg/KO (gray bars), and wild-type C57BL/6 (black bars) mice. α-Gal A activity in hR301Q α-Gal A Tg/KO and GLA KO mice was significantly lower than that of wild-type mice (*P < 0.05, t-test); α-Gal A activity in hR301Q α-Gal A Tg/KO mice was significantly higher than that of GLA KO mice (#P < 0.05, t-test). GL-3 levels in hR301Q α-Gal A Tg/KO and GLA KO mice were significantly higher than those of wild-type mice (*P < 0.05, t-test). Each bar represents the mean ± SEM from four mice per group analyzed in triplicate. (b) Cell-type specific GL-3 staining was assessed by immunohistochemistry in GLA KO, hR301Q α-Gal A Tg/KO, and wild-type C57BL/6 mouse tissues. GL-3 staining is represented as brown spots (black arrows); nuclei are represented as green spots (stained with methyl green). The data shown are representative photomicrographs from four 12-week-old male mice using ×20 magnification, except for the glomeruli, which are representative of four 7-month-old male mice using ×40 magnification. α-Gal A, α-galactosidase A; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.
    Rabbit Antihuman α Gal A Primary Antibody, supplied by Bio-Rad, used in various techniques. Bioz Stars score: 96/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
    https://www.bioz.com/result/rabbit antihuman α gal a primary antibody/product/Bio-Rad
    Average 96 stars, based on 1 article reviews
    rabbit antihuman α gal a primary antibody - by Bioz Stars, 2026-03
    96/100 stars
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    hR301Q α-Gal A Tg/KO mice have reduced α-Gal A and elevated GL-3 levels. (a) α-Gal A and GL-3 levels were assessed in tissue lysates prepared from skin, heart, and kidney of 12-week-old male GLA KO (open bars), hR301Q α-Gal A Tg/KO (gray bars), and wild-type C57BL/6 (black bars) mice. α-Gal A activity in hR301Q α-Gal A Tg/KO and GLA KO mice was significantly lower than that of wild-type mice (*P < 0.05, t-test); α-Gal A activity in hR301Q α-Gal A Tg/KO mice was significantly higher than that of GLA KO mice (#P < 0.05, t-test). GL-3 levels in hR301Q α-Gal A Tg/KO and GLA KO mice were significantly higher than those of wild-type mice (*P < 0.05, t-test). Each bar represents the mean ± SEM from four mice per group analyzed in triplicate. (b) Cell-type specific GL-3 staining was assessed by immunohistochemistry in GLA KO, hR301Q α-Gal A Tg/KO, and wild-type C57BL/6 mouse tissues. GL-3 staining is represented as brown spots (black arrows); nuclei are represented as green spots (stained with methyl green). The data shown are representative photomicrographs from four 12-week-old male mice using ×20 magnification, except for the glomeruli, which are representative of four 7-month-old male mice using ×40 magnification. α-Gal A, α-galactosidase A; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: hR301Q α-Gal A Tg/KO mice have reduced α-Gal A and elevated GL-3 levels. (a) α-Gal A and GL-3 levels were assessed in tissue lysates prepared from skin, heart, and kidney of 12-week-old male GLA KO (open bars), hR301Q α-Gal A Tg/KO (gray bars), and wild-type C57BL/6 (black bars) mice. α-Gal A activity in hR301Q α-Gal A Tg/KO and GLA KO mice was significantly lower than that of wild-type mice (*P < 0.05, t-test); α-Gal A activity in hR301Q α-Gal A Tg/KO mice was significantly higher than that of GLA KO mice (#P < 0.05, t-test). GL-3 levels in hR301Q α-Gal A Tg/KO and GLA KO mice were significantly higher than those of wild-type mice (*P < 0.05, t-test). Each bar represents the mean ± SEM from four mice per group analyzed in triplicate. (b) Cell-type specific GL-3 staining was assessed by immunohistochemistry in GLA KO, hR301Q α-Gal A Tg/KO, and wild-type C57BL/6 mouse tissues. GL-3 staining is represented as brown spots (black arrows); nuclei are represented as green spots (stained with methyl green). The data shown are representative photomicrographs from four 12-week-old male mice using ×20 magnification, except for the glomeruli, which are representative of four 7-month-old male mice using ×40 magnification. α-Gal A, α-galactosidase A; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques: Activity Assay, Staining, Immunohistochemistry, Knock-Out, Transgenic Assay

    DGJ administration increases α-Gal A activity and reduces GL-3 in hR301Q α-Gal A Tg/KO mice. Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 4 weeks at the indicated doses. Lysates from skin, heart, and kidney were subsequently tested for (a) α-Gal A activity and (b,c) GL-3 levels. (a) Significant increases in α-Gal A activity were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent increase in α-Gal A activity (post hoc analysis, P < 0.05). Insets: Tissue α-Gal A protein levels were determined by western blotting. A dose-dependent increase in the 46 kd mature form of α-Gal A was seen in all tissues. Each lane on the blot contains tissue lysate from a single animal. The western blot shown is representative of two experiments. (b) Significant reductions in GL-3 levels were seen in all three tissues as measured by LC-MS/MS (*P < 0.05 versus untreated, t-test). Blue bars (a,b) represent α-Gal A and GL-3 levels from age-matched untreated wild-type C57BL/6 mice. Each bar represents pooled data from three independent studies with the mean ± SEM of 21 hR301Q α-Gal A Tg/KO mice/group or four untreated wild-type mice, all analyzed in triplicate. (c) Cell type–specific reduction of GL-3 in hR301Q α-Gal A Tg/KO mice administered DGJ (100 mg/kg per day) ad libitum in drinking water was assessed by immunohistochemistry. GL-3 staining is represented as brown spots denoted with black arrows. The data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, insets: ×40). α-Gal A, α-galactosidase A; DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: DGJ administration increases α-Gal A activity and reduces GL-3 in hR301Q α-Gal A Tg/KO mice. Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 4 weeks at the indicated doses. Lysates from skin, heart, and kidney were subsequently tested for (a) α-Gal A activity and (b,c) GL-3 levels. (a) Significant increases in α-Gal A activity were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent increase in α-Gal A activity (post hoc analysis, P < 0.05). Insets: Tissue α-Gal A protein levels were determined by western blotting. A dose-dependent increase in the 46 kd mature form of α-Gal A was seen in all tissues. Each lane on the blot contains tissue lysate from a single animal. The western blot shown is representative of two experiments. (b) Significant reductions in GL-3 levels were seen in all three tissues as measured by LC-MS/MS (*P < 0.05 versus untreated, t-test). Blue bars (a,b) represent α-Gal A and GL-3 levels from age-matched untreated wild-type C57BL/6 mice. Each bar represents pooled data from three independent studies with the mean ± SEM of 21 hR301Q α-Gal A Tg/KO mice/group or four untreated wild-type mice, all analyzed in triplicate. (c) Cell type–specific reduction of GL-3 in hR301Q α-Gal A Tg/KO mice administered DGJ (100 mg/kg per day) ad libitum in drinking water was assessed by immunohistochemistry. GL-3 staining is represented as brown spots denoted with black arrows. The data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, insets: ×40). α-Gal A, α-galactosidase A; DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques: Activity Assay, Western Blot, Liquid Chromatography with Mass Spectroscopy, Immunohistochemistry, Staining, Knock-Out, Transgenic Assay

    Long-term (24-week) administration of DGJ results in greater GL-3 reduction compared to 4-week administration. (a) Four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks at the indicated doses. GL-3 levels were subsequently measured in skin, heart, and kidney lysates by LC-MS/MS. Significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent decrease in GL-3 levels (post hoc analysis, P < 0.05). Each bar represents the mean ± SEM of 7–8 mice/dose group analyzed in triplicate. (b) Cell type–specific GL-3 reductions were assessed by immunohistochemistry. GL-3 staining is represented as brown spots. Data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, except glomeruli: ×40). DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: Long-term (24-week) administration of DGJ results in greater GL-3 reduction compared to 4-week administration. (a) Four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks at the indicated doses. GL-3 levels were subsequently measured in skin, heart, and kidney lysates by LC-MS/MS. Significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). The effect was also significant for a linear trend, indicating a dose-dependent decrease in GL-3 levels (post hoc analysis, P < 0.05). Each bar represents the mean ± SEM of 7–8 mice/dose group analyzed in triplicate. (b) Cell type–specific GL-3 reductions were assessed by immunohistochemistry. GL-3 staining is represented as brown spots. Data shown are representative photomicrographs from 7–8 mice/group (magnification: ×20, except glomeruli: ×40). DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques: Liquid Chromatography with Mass Spectroscopy, Immunohistochemistry, Staining, Knock-Out, Transgenic Assay

    Effect of 4- and 24-week DGJ administration on tissue GL-3 levels in hR301Q α-Gal A Tg/KO mice

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: Effect of 4- and 24-week DGJ administration on tissue GL-3 levels in hR301Q α-Gal A Tg/KO mice

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques:

    Less frequent DGJ administration results in greater GL-3 reduction. (a) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered drinking water (red dotted lines) or DGJ [100 mg/kg per day (red solid lines) ad libitum in drinking water] for 4 weeks, followed by a washout period (drinking water only) of up to 7 days. Groups of mice were then euthanized on days 0, 1, 3, 5, or 7 after DGJ withdrawal. Skin, heart, and kidney were collected at each time point, and α-Gal A activity and DGJ levels (blue lines) were measured in tissue lysates. Significantly increased α-Gal A activity was sustained in all three tissues for up to 7 days (*P < 0.05 versus untreated, t-test). For the α-Gal A activity, each data point represents the mean ± SEM of 7–8 mice/time point analyzed in triplicate; data were normalized to untreated levels. For tissue DGJ levels, each data point represents the mean ± SEM for five mice/time point. The limit of quantitation for DGJ in skin, heart, and kidney was 6, 4, and 10 ng/g, respectively. (b) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ (300 mg/kg per day) ad libitum in drinking water for 4 weeks either daily or less frequently using four cycles of a “4 on/3 off” regimen. GL-3 levels were subsequently measured in lysates from skin, heart, and kidney by LC-MS/MS. Significant reductions in GL-3 levels were seen in all tissues (*P < 0.05 versus untreated, t-test). GL-3 levels in skin, heart, and kidney also showed significantly greater reductions with less frequent compared to daily administration (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents pooled data from three independent studies with the mean ± SEM of 21 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: Less frequent DGJ administration results in greater GL-3 reduction. (a) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered drinking water (red dotted lines) or DGJ [100 mg/kg per day (red solid lines) ad libitum in drinking water] for 4 weeks, followed by a washout period (drinking water only) of up to 7 days. Groups of mice were then euthanized on days 0, 1, 3, 5, or 7 after DGJ withdrawal. Skin, heart, and kidney were collected at each time point, and α-Gal A activity and DGJ levels (blue lines) were measured in tissue lysates. Significantly increased α-Gal A activity was sustained in all three tissues for up to 7 days (*P < 0.05 versus untreated, t-test). For the α-Gal A activity, each data point represents the mean ± SEM of 7–8 mice/time point analyzed in triplicate; data were normalized to untreated levels. For tissue DGJ levels, each data point represents the mean ± SEM for five mice/time point. The limit of quantitation for DGJ in skin, heart, and kidney was 6, 4, and 10 ng/g, respectively. (b) Eight-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ (300 mg/kg per day) ad libitum in drinking water for 4 weeks either daily or less frequently using four cycles of a “4 on/3 off” regimen. GL-3 levels were subsequently measured in lysates from skin, heart, and kidney by LC-MS/MS. Significant reductions in GL-3 levels were seen in all tissues (*P < 0.05 versus untreated, t-test). GL-3 levels in skin, heart, and kidney also showed significantly greater reductions with less frequent compared to daily administration (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents pooled data from three independent studies with the mean ± SEM of 21 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques: Activity Assay, Quantitation Assay, Liquid Chromatography with Mass Spectroscopy, Knock-Out, Transgenic Assay

    Long-term DGJ administration reduces accumulated tissue GL-3 in aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. GL-3 levels were measured in lysates of skin, heart, and kidney by LC-MS/MS. Dose-dependent and significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). Reduction of GL-3 in skin and heart was significantly greater after less frequent compared to daily administration of 300 mg/kg DGJ (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: Long-term DGJ administration reduces accumulated tissue GL-3 in aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 24 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. GL-3 levels were measured in lysates of skin, heart, and kidney by LC-MS/MS. Dose-dependent and significant reductions in GL-3 were seen in all three tissues (*P < 0.05 versus untreated, t-test). Reduction of GL-3 in skin and heart was significantly greater after less frequent compared to daily administration of 300 mg/kg DGJ (#P < 0.05 daily versus “4 on/3 off”, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques: Liquid Chromatography with Mass Spectroscopy, Knock-Out, Transgenic Assay

    Effect of DGJ on plasma GL-3 levels in hR301Q α-Gal A Tg/KO mice

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: Effect of DGJ on plasma GL-3 levels in hR301Q α-Gal A Tg/KO mice

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques:

    DGJ increases α-Gal A and decreases GL-3 levels in brain of aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 12 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. Brain α-Gal A and GL-3 levels were subsequently measured. Significant increases in α-Gal A and concomitant reductions in GL-3 were seen with DGJ administration (*P < 0.05 versus untreated, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Journal:

    Article Title: The Pharmacological Chaperone 1-Deoxygalactonojirimycin Reduces Tissue Globotriaosylceramide Levels in a Mouse Model of Fabry Disease

    doi: 10.1038/mt.2009.220

    Figure Lengend Snippet: DGJ increases α-Gal A and decreases GL-3 levels in brain of aged hR301Q α-Gal A Tg/KO mice. Twenty-four-week-old male hR301Q α-Gal A Tg/KO mice were administered DGJ ad libitum in drinking water for 12 weeks either daily or less frequently using a “4 on/3 off” regimen at the indicated doses. Brain α-Gal A and GL-3 levels were subsequently measured. Significant increases in α-Gal A and concomitant reductions in GL-3 were seen with DGJ administration (*P < 0.05 versus untreated, t-test). Each bar represents the mean ± SEM of 7–8 mice/group analyzed in triplicate. DGJ, 1-deoxygalactonojirimycin; GL-3, globotriaosylceramide; KO, knockout; Tg, transgenic.

    Article Snippet: 49 Western blotting of tissue α-Gal A. Tissue lysates (50 µg total protein) were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis on 12% polyacrylamide gels (Bio-Rad, Hercules, CA), transferred to polyvinylidene fluoride membranes (Bio-Rad), and immunoblotted with rabbit antihuman α-Gal A primary antibody (1:1,000 dilution).

    Techniques: Knock-Out, Transgenic Assay