pah enu2 mice Search Results


pah-deficient btbr-pah enu2 (pku) mice  (Taconic Biosciences)
90
Taconic Biosciences pah-deficient btbr-pah enu2 (pku) mice
Effect of PAH and PAL gene transfer on blood Phe and Tyr levels after gene delivery to PAH <t>enu2</t> mice. (A) Schematic of in-vivo study design. PAH and PAH encoding AAV vectors were administered by one-time IV delivery on Day 0. (B) Blood Phe levels were measured in naïve PAH enu2 mice, PAH enu2 mice administered with PAH (1e11 vg) or PAL (4e10, 1e11 or 3e11 vg/mouse) and HET mice at day 9 (baseline) and days 5, 14, 34 and 41 post treatment. Significant reduction in blood Phe levels was observed in both PAH and PAL in PAH enu2 mice as compared to naïve PAH enu2 mice. PAH enu2 PAL treated mice show lower Phe than HET mice while PAH treated mice maintain Phe levels comparable to HET mice. (C) Significant increase in blood Tyr to levels comparable to HET mice were achieved in PAH treated mice but not in PAL treated mice. Two-way ANOVA Mixed effect analysis was performed, ***p < 0.001. (D) Blood Phe continued to remain low in PAH and PAL PAH enu2 mice until the end of the study. (E) Blood Tyr remained normal in PAH PAH enu2 mice till the end of the study. Treatment groups consisted of n = 8–10 animals. Statistics by One-way ANOVA Tukey’s multiple comparison, ***p < 0.001.
Pah Deficient Btbr Pah Enu2 (Pku) Mice, supplied by Taconic Biosciences, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
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Average 90 stars, based on 1 article reviews
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pahenu2 mice  (Charles River Laboratories)
90
Charles River Laboratories pahenu2 mice
Effect of PAH and PAL gene transfer on blood Phe and Tyr levels after gene delivery to PAH <t>enu2</t> mice. (A) Schematic of in-vivo study design. PAH and PAH encoding AAV vectors were administered by one-time IV delivery on Day 0. (B) Blood Phe levels were measured in naïve PAH enu2 mice, PAH enu2 mice administered with PAH (1e11 vg) or PAL (4e10, 1e11 or 3e11 vg/mouse) and HET mice at day 9 (baseline) and days 5, 14, 34 and 41 post treatment. Significant reduction in blood Phe levels was observed in both PAH and PAL in PAH enu2 mice as compared to naïve PAH enu2 mice. PAH enu2 PAL treated mice show lower Phe than HET mice while PAH treated mice maintain Phe levels comparable to HET mice. (C) Significant increase in blood Tyr to levels comparable to HET mice were achieved in PAH treated mice but not in PAL treated mice. Two-way ANOVA Mixed effect analysis was performed, ***p < 0.001. (D) Blood Phe continued to remain low in PAH and PAL PAH enu2 mice until the end of the study. (E) Blood Tyr remained normal in PAH PAH enu2 mice till the end of the study. Treatment groups consisted of n = 8–10 animals. Statistics by One-way ANOVA Tukey’s multiple comparison, ***p < 0.001.
Pahenu2 Mice, supplied by Charles River Laboratories, used in various techniques. Bioz Stars score: 90/100, based on 1 PubMed citations. ZERO BIAS - scores, article reviews, protocol conditions and more
https://www.bioz.com/result/pahenu2 mice/product/Charles River Laboratories
Average 90 stars, based on 1 article reviews
pahenu2 mice - by Bioz Stars, 2026-03
90/100 stars
  Buy from Supplier

Image Search Results


Effect of PAH and PAL gene transfer on blood Phe and Tyr levels after gene delivery to PAH enu2 mice. (A) Schematic of in-vivo study design. PAH and PAH encoding AAV vectors were administered by one-time IV delivery on Day 0. (B) Blood Phe levels were measured in naïve PAH enu2 mice, PAH enu2 mice administered with PAH (1e11 vg) or PAL (4e10, 1e11 or 3e11 vg/mouse) and HET mice at day 9 (baseline) and days 5, 14, 34 and 41 post treatment. Significant reduction in blood Phe levels was observed in both PAH and PAL in PAH enu2 mice as compared to naïve PAH enu2 mice. PAH enu2 PAL treated mice show lower Phe than HET mice while PAH treated mice maintain Phe levels comparable to HET mice. (C) Significant increase in blood Tyr to levels comparable to HET mice were achieved in PAH treated mice but not in PAL treated mice. Two-way ANOVA Mixed effect analysis was performed, ***p < 0.001. (D) Blood Phe continued to remain low in PAH and PAL PAH enu2 mice until the end of the study. (E) Blood Tyr remained normal in PAH PAH enu2 mice till the end of the study. Treatment groups consisted of n = 8–10 animals. Statistics by One-way ANOVA Tukey’s multiple comparison, ***p < 0.001.

Journal: Scientific Reports

Article Title: Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria

doi: 10.1038/s41598-021-02267-2

Figure Lengend Snippet: Effect of PAH and PAL gene transfer on blood Phe and Tyr levels after gene delivery to PAH enu2 mice. (A) Schematic of in-vivo study design. PAH and PAH encoding AAV vectors were administered by one-time IV delivery on Day 0. (B) Blood Phe levels were measured in naïve PAH enu2 mice, PAH enu2 mice administered with PAH (1e11 vg) or PAL (4e10, 1e11 or 3e11 vg/mouse) and HET mice at day 9 (baseline) and days 5, 14, 34 and 41 post treatment. Significant reduction in blood Phe levels was observed in both PAH and PAL in PAH enu2 mice as compared to naïve PAH enu2 mice. PAH enu2 PAL treated mice show lower Phe than HET mice while PAH treated mice maintain Phe levels comparable to HET mice. (C) Significant increase in blood Tyr to levels comparable to HET mice were achieved in PAH treated mice but not in PAL treated mice. Two-way ANOVA Mixed effect analysis was performed, ***p < 0.001. (D) Blood Phe continued to remain low in PAH and PAL PAH enu2 mice until the end of the study. (E) Blood Tyr remained normal in PAH PAH enu2 mice till the end of the study. Treatment groups consisted of n = 8–10 animals. Statistics by One-way ANOVA Tukey’s multiple comparison, ***p < 0.001.

Article Snippet: A colony of PAH-deficient BTBR-PAH enu2 (PKU) mice was maintained at Taconic .

Techniques: In Vivo, Comparison

Brain amino acid levels post PAH or PAL administration. (A) Brain Phe is reduced to normal levels in PAH or PAL treated PAH enu2 mice. (B) Brain Tyr levels are restored to normal in PAH PAH enu2 mice but not in PAL PAH enu2 mice. (C) Brain Trp is increased in both PAH and PAL treated PAH enu2 mice. N = 5 per group were used for analysis. Control mice and mice treated with 1e11 vg/mouse of PAH or PAL vector were terminated on day 41 and perfused with PBS. Naïve untreated PAH enu2 mice, PAH or PAL , treated PAH enu2 mice, HET untreated HET mice. One-way ANOVA Tukey’s multiple comparison, **p < 0.01 and ****p < 0.0001.

Journal: Scientific Reports

Article Title: Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria

doi: 10.1038/s41598-021-02267-2

Figure Lengend Snippet: Brain amino acid levels post PAH or PAL administration. (A) Brain Phe is reduced to normal levels in PAH or PAL treated PAH enu2 mice. (B) Brain Tyr levels are restored to normal in PAH PAH enu2 mice but not in PAL PAH enu2 mice. (C) Brain Trp is increased in both PAH and PAL treated PAH enu2 mice. N = 5 per group were used for analysis. Control mice and mice treated with 1e11 vg/mouse of PAH or PAL vector were terminated on day 41 and perfused with PBS. Naïve untreated PAH enu2 mice, PAH or PAL , treated PAH enu2 mice, HET untreated HET mice. One-way ANOVA Tukey’s multiple comparison, **p < 0.01 and ****p < 0.0001.

Article Snippet: A colony of PAH-deficient BTBR-PAH enu2 (PKU) mice was maintained at Taconic .

Techniques: Control, Plasmid Preparation, Comparison

Restoration of brain neurotransmitter levels after PAH or PAL administration. (A) Schematic of major steps involved in synthesis of neurotransmitters dopamine and norepinephrine. ( B) Dopamine precursor L-DOPA was significantly higher in PAL PAH enu2 and HET brains as compared to the naïve PAH enu2 brains. Significant increases in (C) dopamine and (D) norepinephrine were observed in both the PAH and PAL PAH enu2 mouse brains as compared to the naïve mice. (E) Schematic of serotonin synthesis and downstream metabolite 5-hydroxyindolacetic acid (5-HIAA). Serotonin precursor 5-hydroxytryptophan (F) , serotonin (G) and 5-HIAA (H) were all restored to levels similar to HET mice after PAH and PAL administration. N = 5 per group were used for analysis. Control mice and mice treated with 1e11 vg/mouse of PAH or PAL vector were terminated on day 41 and perfused with PBS. Group abbreviations: PKU naïve, untreated PAH enu2 mice; PKU PAH or PAL, treated PAH enu2 mice; HET, untreated HET mice. One-way ANOVA Tukey’s multiple comparison, *p < 0.05, **p < 0.01, ***p < 0.001 and ****p < 0.0001.

Journal: Scientific Reports

Article Title: Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria

doi: 10.1038/s41598-021-02267-2

Figure Lengend Snippet: Restoration of brain neurotransmitter levels after PAH or PAL administration. (A) Schematic of major steps involved in synthesis of neurotransmitters dopamine and norepinephrine. ( B) Dopamine precursor L-DOPA was significantly higher in PAL PAH enu2 and HET brains as compared to the naïve PAH enu2 brains. Significant increases in (C) dopamine and (D) norepinephrine were observed in both the PAH and PAL PAH enu2 mouse brains as compared to the naïve mice. (E) Schematic of serotonin synthesis and downstream metabolite 5-hydroxyindolacetic acid (5-HIAA). Serotonin precursor 5-hydroxytryptophan (F) , serotonin (G) and 5-HIAA (H) were all restored to levels similar to HET mice after PAH and PAL administration. N = 5 per group were used for analysis. Control mice and mice treated with 1e11 vg/mouse of PAH or PAL vector were terminated on day 41 and perfused with PBS. Group abbreviations: PKU naïve, untreated PAH enu2 mice; PKU PAH or PAL, treated PAH enu2 mice; HET, untreated HET mice. One-way ANOVA Tukey’s multiple comparison, *p < 0.05, **p < 0.01, ***p < 0.001 and ****p < 0.0001.

Article Snippet: A colony of PAH-deficient BTBR-PAH enu2 (PKU) mice was maintained at Taconic .

Techniques: Control, Plasmid Preparation, Comparison

Improvement in nesting behavior in PAH and PAL PAH enu2 mice. (A) Baseline behavior measured 2 days prior to treatment and (B) improved at day 34 post PAH and PAL treatment. (C) Tracking the nesting score of individual mice shows that most PAH and PAL treated mice perform better and receive a higher score in the post treatment nesting assay. Treatment groups consisted of n = 8–10 animals. Group abbreviations: PKU naïve, untreated PAH enu2 mice; PKU PAH or PAL, treated PAH enu2 mice; HET, untreated HET mice. Statistics by One-way ANOVA Kruskal–Wallis test followed by Dunn’s multiple comparison, *p < 0.05 and ***p < 0.001.

Journal: Scientific Reports

Article Title: Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria

doi: 10.1038/s41598-021-02267-2

Figure Lengend Snippet: Improvement in nesting behavior in PAH and PAL PAH enu2 mice. (A) Baseline behavior measured 2 days prior to treatment and (B) improved at day 34 post PAH and PAL treatment. (C) Tracking the nesting score of individual mice shows that most PAH and PAL treated mice perform better and receive a higher score in the post treatment nesting assay. Treatment groups consisted of n = 8–10 animals. Group abbreviations: PKU naïve, untreated PAH enu2 mice; PKU PAH or PAL, treated PAH enu2 mice; HET, untreated HET mice. Statistics by One-way ANOVA Kruskal–Wallis test followed by Dunn’s multiple comparison, *p < 0.05 and ***p < 0.001.

Article Snippet: A colony of PAH-deficient BTBR-PAH enu2 (PKU) mice was maintained at Taconic .

Techniques: Comparison

Gene expression changes in livers of PAH enu2 mice. (A) Number of differentially expressed genes in naïve, PAH and PAL treated PAH enu2 mice as compared to HET mice (HET). (B) Venn diagram showing number of DEG common and unique to each group. The majority of DEG in PAH enu2 mice are corrected after PAH or PAL treatment. (C ) Top 10 pathways affected in the liver of PAH enu2 mice as compared to HET mice analyzed by Ingenuity pathway analysis. Superpathway of cholesterol biosynthesis was the most affected pathway in the liver of PAH enu2 mice. (D) Heatmap showing the expression of genes involved in multistep cholesterol biosynthesis pathway. Most genes involved in cholesterol biosynthesis are upregulated in untreated PAH enu2 mice (PKU naive). The expression profile of PAH and PAL treated mice shares similarity to naïve HET mice (HET, control). Heatmap was generated using QIAGEN Omicsoft studio version 10.2.4.7. (E) Top 6 upregulated and downregulated genes in PAH enu2 mice as compared to naïve HET mice. (F) Modest increase in expression of PPARα, activator of Cyp4a14 and FAT, a downstream effector of high Cyp4a14 in PAH enu2 mice. Upregulation of Cyp4a14, PPARα and FAT is corrected in both PAH and PAL treated mice. N = 3 per group were used for analysis. One-way ANOVA Tukey’s multiple comparison, *p < 0.05 and **p < 0.01.

Journal: Scientific Reports

Article Title: Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria

doi: 10.1038/s41598-021-02267-2

Figure Lengend Snippet: Gene expression changes in livers of PAH enu2 mice. (A) Number of differentially expressed genes in naïve, PAH and PAL treated PAH enu2 mice as compared to HET mice (HET). (B) Venn diagram showing number of DEG common and unique to each group. The majority of DEG in PAH enu2 mice are corrected after PAH or PAL treatment. (C ) Top 10 pathways affected in the liver of PAH enu2 mice as compared to HET mice analyzed by Ingenuity pathway analysis. Superpathway of cholesterol biosynthesis was the most affected pathway in the liver of PAH enu2 mice. (D) Heatmap showing the expression of genes involved in multistep cholesterol biosynthesis pathway. Most genes involved in cholesterol biosynthesis are upregulated in untreated PAH enu2 mice (PKU naive). The expression profile of PAH and PAL treated mice shares similarity to naïve HET mice (HET, control). Heatmap was generated using QIAGEN Omicsoft studio version 10.2.4.7. (E) Top 6 upregulated and downregulated genes in PAH enu2 mice as compared to naïve HET mice. (F) Modest increase in expression of PPARα, activator of Cyp4a14 and FAT, a downstream effector of high Cyp4a14 in PAH enu2 mice. Upregulation of Cyp4a14, PPARα and FAT is corrected in both PAH and PAL treated mice. N = 3 per group were used for analysis. One-way ANOVA Tukey’s multiple comparison, *p < 0.05 and **p < 0.01.

Article Snippet: A colony of PAH-deficient BTBR-PAH enu2 (PKU) mice was maintained at Taconic .

Techniques: Gene Expression, Expressing, Control, Generated, Comparison

Changes in protein levels in livers of PAH enu2 mice. (A) Number of differentially expressed proteins in naïve, PAH and PAL treated PAH enu2 mice as compared to HET mice. (B) Top 10 pathways affected in the livers of PAH enu2 mice as compared to HET mice analyzed by Ingenuity pathway analysis. Pathways linked to lipid metabolism are the most affected pathways in the liver of PAH enu2 mice. (C) Top 6 upregulated and downregulated proteins in PAH enu2 mice as compared to HET mice. (D) Network analysis identified Lipid metabolism as the top network in the dataset with Cyp4a11 (Cyp4a10 in mouse) at the bottom of the hierarchy. Network analysis figure was generated using Ingenuity Pathway analysis (IPA) (QIAGEN Inc., https://www.qiagenbioinformatics.com/products/ingenuitypathway-analysis ).

Journal: Scientific Reports

Article Title: Blood phenylalanine reduction reverses gene expression changes observed in a mouse model of phenylketonuria

doi: 10.1038/s41598-021-02267-2

Figure Lengend Snippet: Changes in protein levels in livers of PAH enu2 mice. (A) Number of differentially expressed proteins in naïve, PAH and PAL treated PAH enu2 mice as compared to HET mice. (B) Top 10 pathways affected in the livers of PAH enu2 mice as compared to HET mice analyzed by Ingenuity pathway analysis. Pathways linked to lipid metabolism are the most affected pathways in the liver of PAH enu2 mice. (C) Top 6 upregulated and downregulated proteins in PAH enu2 mice as compared to HET mice. (D) Network analysis identified Lipid metabolism as the top network in the dataset with Cyp4a11 (Cyp4a10 in mouse) at the bottom of the hierarchy. Network analysis figure was generated using Ingenuity Pathway analysis (IPA) (QIAGEN Inc., https://www.qiagenbioinformatics.com/products/ingenuitypathway-analysis ).

Article Snippet: A colony of PAH-deficient BTBR-PAH enu2 (PKU) mice was maintained at Taconic .

Techniques: Generated