mps vi mice model  (Jackson Laboratory)

Journal: Molecular Genetics and Metabolism Reports

Article Title: Reduction of lysosome abundance and GAG accumulation after odiparcil treatment in MPS I and MPS VI models

doi: 10.1016/j.ymgmr.2023.101011

Figure Lengend Snippet: Lysotracker visualization of lysosomes in MPS I and MPS VI fibroblasts and effect of odiparcil treatment. A. Representative images of lysotracker labelling in healthy (control) and MPS I fibroblasts. B, C. Quantification of fluorescence of lysotracker in control and MPS I (B) or MPS VI (C) fibroblasts. D, E. Representative images of MPS VI (D) or MPS I (E) fibroblasts treated with vehicle (DMSO) or odiparcil. F, G. Quantification of fluorescence of lysotracker after odiparcil treatment in MPS VI (F) or MPS I (G) fibroblasts, normalized to the DMSO vehicle control. Data is presented as mean ± SEM. Groups were statistically analysed using a one-way ANOVA followed by Dunnett's test; * p < 0.05, ** p < 0.01, *** p < 0.001 compared to non-affected or DMSO controls (as indicated).

Article Snippet: Male MPS I mice model [ ] ( Idua tm1.1Kmke allele homozygous, referred to as Idua − ; note we observed visibly stronger MPS I appearance in the male mice) and male and female MPS VI mice model [ , ] [ , ] ( Arsb m1J allele homozygous, referred to as Arsb − ) and wildtype (WT) littermates were obtained from internal breeding of stock mice derived from The Jackson Laboratory (MPS I model: strain No: 017681; B6.129S- Idua tm1.1Kmke /J and MPS VI model: strain No: 005598; C57BL/6 J- Arsb m1J /GrsrJ).

Techniques: Control, Fluorescence

Journal: Molecular Genetics and Metabolism Reports

Article Title: Reduction of lysosome abundance and GAG accumulation after odiparcil treatment in MPS I and MPS VI models

doi: 10.1016/j.ymgmr.2023.101011

Figure Lengend Snippet: GAG Quantification in Tissues from MPS VI and MPS I mice treated with odiparcil. A, B. CS, DS and HS quantification in extracts from liver (A) and eye (B) of WT control, Arsb- control and Arsb- mice treated with the indicated dose of odiparcil (g odiparcil per kg diet); C, D. CS, DS and HS quantification in extracts from liver (C) and eye (D) of WT control, Idua- control and Idua- mice treated with the indicated dose of odiparcil (g odiparcil per kg diet); Data is presented as mean ± SEM. Groups were statistically analysed using a one-way ANOVA followed by Dunnett's test; ** p < 0.01, *** p < 0.001 compared to WT or Arsb − or Idua- controls (as indicated).

Article Snippet: Male MPS I mice model [ ] ( Idua tm1.1Kmke allele homozygous, referred to as Idua − ; note we observed visibly stronger MPS I appearance in the male mice) and male and female MPS VI mice model [ , ] [ , ] ( Arsb m1J allele homozygous, referred to as Arsb − ) and wildtype (WT) littermates were obtained from internal breeding of stock mice derived from The Jackson Laboratory (MPS I model: strain No: 017681; B6.129S- Idua tm1.1Kmke /J and MPS VI model: strain No: 005598; C57BL/6 J- Arsb m1J /GrsrJ).

Techniques: Control

Journal: PLoS ONE

Article Title: Odiparcil, a potential glycosaminoglycans clearance therapy in mucopolysaccharidosis VI—Evidence from in vitro and in vivo models

doi: 10.1371/journal.pone.0233032

Figure Lengend Snippet: Total GAG accumulation at 1, 3 and 6 months of age in liver (A) and kidney (B). Above bars for Arsb - shown percent increase in total GAG compared to WT siblings at the same age. Data represented as mean ± SEM;**: p value < 0.01; ***: p value < 0.001.

Article Snippet: MPS VI model nonsense mutation Arsb m1J homozygous mice [ , ] (referred to as Arsb - throughout the manuscript) and WT littermates were obtained from internal breeding of stock mice derived from The Jackson Laboratory strain No: 005598 (C57BL/6J-Arsb m1J /GrsrJ).

Techniques:

Journal: PLoS ONE

Article Title: Odiparcil, a potential glycosaminoglycans clearance therapy in mucopolysaccharidosis VI—Evidence from in vitro and in vivo models

doi: 10.1371/journal.pone.0233032

Figure Lengend Snippet: Effect of odiparcil treatment on Alcian Blue staining in liver and kidney of Arsb - mice in the early disease model (A) and in the advanced disease model (B). Data represented as mean ± SEM;*: p-value<0.05, **: p value < 0.01; ***: p value < 0.001.

Article Snippet: MPS VI model nonsense mutation Arsb m1J homozygous mice [ , ] (referred to as Arsb - throughout the manuscript) and WT littermates were obtained from internal breeding of stock mice derived from The Jackson Laboratory strain No: 005598 (C57BL/6J-Arsb m1J /GrsrJ).

Techniques: Staining

Journal: PLoS ONE

Article Title: Odiparcil, a potential glycosaminoglycans clearance therapy in mucopolysaccharidosis VI—Evidence from in vitro and in vivo models

doi: 10.1371/journal.pone.0233032

Figure Lengend Snippet: Dose dependency of odiparcil treatment on cartilage thickening in trachea and femoral growth plate of Arsb - mice in the early disease model (A) and in the established disease model (B). Data represented as mean ± SEM;*: p-value<0.05, **: p value < 0.01; ***: p value < 0.001.

Article Snippet: MPS VI model nonsense mutation Arsb m1J homozygous mice [ , ] (referred to as Arsb - throughout the manuscript) and WT littermates were obtained from internal breeding of stock mice derived from The Jackson Laboratory strain No: 005598 (C57BL/6J-Arsb m1J /GrsrJ).

Techniques:

Journal: PLoS ONE

Article Title: Odiparcil, a potential glycosaminoglycans clearance therapy in mucopolysaccharidosis VI—Evidence from in vitro and in vivo models

doi: 10.1371/journal.pone.0233032

Figure Lengend Snippet: Only blood smears from non-treated controls and high dose odiparcil treated Arsb - were analysed. Data represented as mean ± SEM; ns: p value > 0.05, **:— p value < 0.01; ***: p value < 0.001.

Article Snippet: MPS VI model nonsense mutation Arsb m1J homozygous mice [ , ] (referred to as Arsb - throughout the manuscript) and WT littermates were obtained from internal breeding of stock mice derived from The Jackson Laboratory strain No: 005598 (C57BL/6J-Arsb m1J /GrsrJ).

Techniques: