INT346581

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Context Info
Confidence 0.25
First Reported 2010
Last Reported 2010
Negated 0
Speculated 0
Reported most in Body
Documents 1
Total Number 17
Disease Relevance 4.08
Pain Relevance 0.11

This is a graph with borders and nodes. Maybe there is an Imagemap used so the nodes may be linking to some Pages.

lipid binding (Sh3gl3) endosome (Sh3gl3) cytoplasm (Sh3gl3)
Anatomy Link Frequency
kidney 4
urine 3
visceral 1
liver 1
spleen 1
Sh3gl3 (Mus musculus)
Pain Link Frequency Relevance Heat
hypoalgesia 17 59.92 Quite High
peripheral neuropathy 51 45.28 Quite Low
Spinal cord 34 5.00 Very Low Very Low Very Low
Neuropathic pain 34 5.00 Very Low Very Low Very Low
Peripheral nervous system 34 5.00 Very Low Very Low Very Low
anesthesia 17 5.00 Very Low Very Low Very Low
analgesia 17 5.00 Very Low Very Low Very Low
isoflurane 17 5.00 Very Low Very Low Very Low
Disease Link Frequency Relevance Heat
Fabry Disease 306 100.00 Very High Very High Very High
Proteinuria 17 99.04 Very High Very High Very High
Gauchers Disease 119 96.88 Very High Very High Very High
Renal Disease 17 91.52 High High
Chronic Renal Failure 17 88.96 High High
Disease 187 88.68 High High
Death 17 86.40 High High
Polyuria 17 81.92 Quite High
Renal Failure 17 79.28 Quite High
Disease Progression 34 78.08 Quite High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
Visceral tissues (liver, kidney, heart and spleen), whole blood and urine all demonstrated elevated levels of GL-3 in the Fabry-Rag mouse relative to the wild-type controls at all time points (data not shown).
Gene_expression (levels) of GL-3 in heart
1) Confidence 0.25 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0 Pain Relevance 0
Combined ERT and SRT is most effective at reducing GL-3 levels in tissues of the Fabry-Rag mouse
Gene_expression (levels) of GL-3
2) Confidence 0.25 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.06 Pain Relevance 0.06
Figure 4A shows that although all treatment paradigms reduced GL-3, the greatest and most consistent reductions regardless of tissue were obtained using infusions of ?
Gene_expression (reduced) of GL-3
3) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0 Pain Relevance 0
SRT normalizes urine volume and decreases urine GL-3 levels
Gene_expression (levels) of GL-3 in urine
4) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.60 Pain Relevance 0
Overall, these results are consistent with an ability of eliglustat tartrate to inhibit (to some degree) the synthesis of glycosphingolipids such as GL-3, but because this inhibition is not complete and given the total absence of ?
Gene_expression (synthesis) of GL-3
5) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.32 Pain Relevance 0
In some assays, notably kidney and urine GL-3 levels (Figure 4), urine uromodulin level (Figure 5), heat-sensitivity (Figure 6) and degree of DRG vacuolation (Figure 7), this therapeutic option was as, or more effective than, ERT every two months alone.
Gene_expression (levels) of GL-3 in urine
6) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.25 Pain Relevance 0
Treated Fabry mice also continued to accumulate GL-3 throughout the course of the study, but did so at a reduced rate, generally resulting in significant net GL-3 reductions of 40–50% by the end of the study.
Gene_expression (reductions) of GL-3
7) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0 Pain Relevance 0
Treating Fabry mice with eliglustat tartrate (SRT) reduced the urine GL-3 concentration to about 50% that of the untreated mice, mirroring the benefit observed in the visceral tissues (Figure 1).
Gene_expression (concentration) of GL-3 in visceral
8) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.57 Pain Relevance 0
SRT works on the principle of limiting the production of the pathologic substrate, which in the case of Fabry disease is primarily GL-3.
Gene_expression (production) of GL-3 associated with fabry disease
9) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.56 Pain Relevance 0
Together with the data in Figure 1 showing GL-3 deposition in the kidney over time, these results demonstrate renal involvement in the Fabry mouse with physiologic consequences that may mirror the early stages of kidney pathophysiology in Fabry patients.
Gene_expression (deposition) of GL-3 in kidney
10) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.18 Pain Relevance 0
In untreated Fabry patients, GL-3 deposition in the kidney leads to proteinuria and progressively decreasing glomerular filtration rates.
Gene_expression (deposition) of GL-3 in kidney associated with proteinuria
11) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.63 Pain Relevance 0
20-fold increase in urine GL-3 levels in the untreated Fabry mice relative to that of age-matched wild-type controls.
Gene_expression (levels) of GL-3 in urine
12) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.63 Pain Relevance 0
The combination of ERT and SRT was evaluated in 3-month old Fabry-Rag mice, an age at which significant GL-3 has accumulated; absolute GL-3 levels were comparable between Fabry and Fabry-Rag mice (data not shown).
Gene_expression (levels) of GL-3
13) Confidence 0.22 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.05 Pain Relevance 0.05
This suggests that the source of the urine GL-3 is not a representative blood filtrate but more likely derived from the kidney.
Gene_expression (source) of GL-3 in kidney
14) Confidence 0.20 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0.23 Pain Relevance 0
Visceral tissues (liver, kidney, heart and spleen), whole blood and urine all demonstrated elevated levels of GL-3 in the Fabry-Rag mouse relative to the wild-type controls at all time points (data not shown).
Gene_expression (levels) of GL-3 in spleen
15) Confidence 0.09 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0 Pain Relevance 0
Visceral tissues (liver, kidney, heart and spleen), whole blood and urine all demonstrated elevated levels of GL-3 in the Fabry-Rag mouse relative to the wild-type controls at all time points (data not shown).
Gene_expression (levels) of GL-3 in liver
16) Confidence 0.09 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0 Pain Relevance 0
Visceral tissues (liver, kidney, heart and spleen), whole blood and urine all demonstrated elevated levels of GL-3 in the Fabry-Rag mouse relative to the wild-type controls at all time points (data not shown).
Gene_expression (levels) of GL-3 in kidney
17) Confidence 0.09 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2991350 Disease Relevance 0 Pain Relevance 0

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