INT210060

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Context Info
Confidence 0.59
First Reported 2007
Last Reported 2008
Negated 0
Speculated 0
Reported most in Body
Documents 4
Total Number 18
Disease Relevance 6.61
Pain Relevance 1.37

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) signal transduction (SH3GL3)
cytoplasm (SH3GL3)
Anatomy Link Frequency
kidney 3
endothelium 1
podocytes 1
parenchyma 1
body 1
SH3GL3 (Homo sapiens)
Pain Link Frequency Relevance Heat
Pain score 22 98.28 Very High Very High Very High
ischemia 17 96.36 Very High Very High Very High
Pain 68 95.92 Very High Very High Very High
Central nervous system 77 91.32 High High
tolerance 30 65.76 Quite High
cva 36 52.96 Quite High
Neuropathic pain 15 51.44 Quite High
fibrosis 90 40.88 Quite Low
headache 1 26.68 Quite Low
imagery 6 5.00 Very Low Very Low Very Low
Disease Link Frequency Relevance Heat
Fabry Disease 692 99.40 Very High Very High Very High
Pain 90 98.28 Very High Very High Very High
Disease 420 97.56 Very High Very High Very High
Cv Unclassified Under Development 17 96.36 Very High Very High Very High
Angiokeratoma 40 94.60 High High
Hypohidrosis 25 94.00 High High
Diarrhoea 5 92.56 High High
Renal Disease 652 92.32 High High
Proteinuria 1284 88.28 High High
Stress 15 87.92 High High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
Long-term follow-up is needed for the evaluation of effects on urinary GL-3 excretion in this patient.
Localization (excretion) of GL-3
1) Confidence 0.59 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 1.11 Pain Relevance 0.32
Urinary excretion of GL-3 has not been reduced except in patient 4, although his renal function has remained stable during ERT.
Localization (excretion) of GL-3
2) Confidence 0.55 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 0.88 Pain Relevance 0.26
Measurements of tissue levels of GL-3 are invasive, so more readily accessible samples, such as urinary GL-3 excretion are obviously desirable.
Localization (excretion) of GL-3
3) Confidence 0.22 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.34 Pain Relevance 0
The increase in urinary GL-3 excretion above baseline in antibody-positive patients treated with ERT at 0.2 mg/kg every other week only became apparent after 6 months of infusion therapy, was not correlated with infusion-related reactions.
Localization (excretion) of GL-3
4) Confidence 0.20 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0 Pain Relevance 0
D) Do short-term changes in urinary GL-3 excretion reflect changes in the total body GL-3 load, or just changes in renal GL-3?
Localization (excretion) of GL-3 in body
5) Confidence 0.20 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.20 Pain Relevance 0.04
As shown in Figure 8, there is a relationship between the amount of GL-3 in the kidney, as assessed by determinations of GL-3 content in kidney biopsy specimens, and the severity of glomerular damage (Figure 8A), as well as the urinary excretion of GL-3 (Figure 8B).
Localization (excretion) of GL-3 in kidney
6) Confidence 0.19 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.38 Pain Relevance 0
In all patients, light and electron microscopy showed severe GL-3 accumulation in podocytes.
Localization (accumulation) of GL-3 in podocytes
7) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.40 Pain Relevance 0
C) Are short-term changes in urinary GL-3 excretion of any utility is assessing response to ERT therapy?
Localization (excretion) of GL-3
8) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.21 Pain Relevance 0.03
Measurements of tissue levels of GL-3 are invasive, so more readily accessible samples, such as urinary GL-3 excretion are obviously desirable.
Localization (excretion) of GL-3
9) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.35 Pain Relevance 0
Secondary outcome measures included reduction of microvascular endothelial deposits in heart, kidney and skin, change in baseline urinary GL-3 excretion and kidney GL-3 content, and reduction in pain scores.
Localization (excretion) of GL-3 in kidney associated with pain score
10) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.10 Pain Relevance 0.10
Urinary GL-3 decreased by 29% in the active-treatment group, and increased by 15.4% in the placebo-treated patients.


Localization (decreased) of GL-3
11) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.36 Pain Relevance 0.10
With time, progressive GL-3 accumulation in the microvasculature and parenchyma leads to microvascular dysfunction, occlusion, and ischemia.
Localization (accumulation) of GL-3 in parenchyma associated with ischemia
12) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 1.07 Pain Relevance 0.14
If there is a progressive rise in urinary GL-3 excretion after several months of ERT, then consideration should be given to increasing the frequency of monitoring of clinical outcomes.
Localization (excretion) of GL-3
13) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.08 Pain Relevance 0.03
The overall composite score for microvascular endothelial GL-3 accumulation decreased from 4.9 ± 1.5 at baseline to 0.7 ± 0.8 at week 20 (85.7% decrease).
Localization (accumulation) of GL-3
14) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.10 Pain Relevance 0.10
As shown in Figure 8, there is a relationship between the amount of GL-3 in the kidney, as assessed by determinations of GL-3 content in kidney biopsy specimens, and the severity of glomerular damage (Figure 8A), as well as the urinary excretion of GL-3 (Figure 8B).
Localization (excretion) of GL-3 in kidney
15) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.40 Pain Relevance 0
Therefore, reduction in GL-3 inclusions did not predict clinical efficacy, and therefore resolution of GL-3 did not fulfill the criteria for a validated surrogate marker (Fleming 2005).
Localization (resolution) of GL-3
16) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.13 Pain Relevance 0.05
For now, it would seem prudent to regularly monitor urinary GL-3 excretion, especially in patients treated with ERT at 0.2 mg/kg every 2 weeks.
Localization (excretion) of GL-3
17) Confidence 0.18 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.07 Pain Relevance 0.03
The first placebo controlled trial with agalsidase beta focused mainly on surrogate endpoints, including clearance of GL-3 from renal endothelium as well as a reduction in GL-3 accumulation in the heart, skin and plasma [6].
Localization (clearance) of GL-3 in endothelium
18) Confidence 0.06 Published 2007 Journal PLoS ONE Section Body Doc Link PMC1913555 Disease Relevance 0.44 Pain Relevance 0.17

General Comments

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