INT142724

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Context Info
Confidence 0.53
First Reported 2007
Last Reported 2010
Negated 0
Speculated 0
Reported most in Body
Documents 11
Total Number 14
Disease Relevance 8.23
Pain Relevance 2.61

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

transferase activity, transferring glycosyl groups (A4GALT) Golgi apparatus (A4GALT) plasma membrane organization (A4GALT)
Anatomy Link Frequency
plasma 6
urine 2
endothelial cells 1
skin 1
A4GALT (Homo sapiens)
Pain Link Frequency Relevance Heat
Pain 175 99.32 Very High Very High Very High
Neuropathic pain 38 97.76 Very High Very High Very High
Potency 4 95.60 Very High Very High Very High
Lasting pain 5 89.04 High High
Bioavailability 2 87.44 High High
fibrosis 57 78.88 Quite High
palliative 14 65.80 Quite High
Pain score 8 64.08 Quite High
imagery 112 57.48 Quite High
aspirin 24 35.80 Quite Low
Disease Link Frequency Relevance Heat
Fabry Disease 1223 99.76 Very High Very High Very High
Pain 200 99.32 Very High Very High Very High
Neuropathic Pain 50 97.76 Very High Very High Very High
Aging 2 97.36 Very High Very High Very High
Stroke 108 95.28 Very High Very High Very High
Chronic Renal Failure 108 95.16 Very High Very High Very High
Coronary Heart Disease 67 94.80 High High
Congenital Anomalies 94 94.68 High High
Atherosclerosis 6 85.92 High High
Hypohidrosis 12 85.04 High High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
Enzyme replacement therapy (ERT) with intravenous infusions of recombinant human alpha-galactosidase A consistently decreases Gb3 levels in plasma and clears lysosomal inclusions from vascular endothelial cells.
Negative_regulation (decreases) of Gb3 in endothelial cells
1) Confidence 0.53 Published 2007 Journal Ann. Intern. Med. Section Abstract Doc Link 17371887 Disease Relevance 0.66 Pain Relevance 0.09
Studies with both preparations have described a reduction in plasma, urinary sediment and tissue levels of Gb3, a decrease in the frequency of pain crisis and a reduction in left ventricular mass and improvement or stabilization of renal function.
Negative_regulation (reduction) of Gb3 in plasma associated with pain
2) Confidence 0.35 Published 2010 Journal Curr. Med. Chem. Section Abstract Doc Link 20345350 Disease Relevance 0.93 Pain Relevance 0.14
There, ERT resulted in a decrease of Gb3-accumulation in skin biopsies and improvement of gastrointestinal symptoms after 24 weeks treatment.
Negative_regulation (decrease) of Gb3 in skin
3) Confidence 0.21 Published 2009 Journal Orphanet J Rare Dis Section Body Doc Link PMC2768700 Disease Relevance 0.23 Pain Relevance 0
Though reduction of myocardial Gb3-content did not reach significance, LVM as measured by cardiac MRI and echocardiography was significantly decreased in the patients treated with Agalsidase alfa compared to an increase in the placebo group.
Negative_regulation (reduction) of Gb3
4) Confidence 0.21 Published 2009 Journal Orphanet J Rare Dis Section Body Doc Link PMC2768700 Disease Relevance 0.95 Pain Relevance 0.06
In the 3.5-year extension study of one of the pediatric clinical trials, there were sustained, statistically significant improvements in the clinical features of FD, including reduced plasma Gb3 levels, reduced pain severity assessed by the brief pain inventory (BPI) questionnaire, and improved heart rate variability.
Negative_regulation (reduced) of Gb3 in plasma associated with pain and fabry disease
5) Confidence 0.14 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.77 Pain Relevance 0.41
In two pediatric clinical trials of ERT with agalsidase alfa, including 37 children [342,343], boys demonstrated reductions in plasma Gb3 levels, and both boys and girls reported reductions in neuropathic pain and in the use of neuropathic pain medications.
Negative_regulation (reductions) of Gb3 in plasma associated with neuropathic pain
6) Confidence 0.14 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.60 Pain Relevance 0.19
In the phase III pivotal study, agalsidase alfa treatment resulted in a 54% reduction in plasma Gb3 levels from baseline, as measured by HPLC.
Negative_regulation (reduction) of Gb3 in plasma
7) Confidence 0.13 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.89 Pain Relevance 0.56
Mean urine Gb3 levels were reduced to normal range.
Negative_regulation (reduced) of Gb3 in urine
8) Confidence 0.13 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.77 Pain Relevance 0.46
Infusion of a dose of 1.0 mg/kg resulted in a more robust decline in Gb3, less impact of antibodies, stable renal function and reduction of LVM [337].
Negative_regulation (decline) of Gb3
9) Confidence 0.13 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.22 Pain Relevance 0
Moreover, no studies have reported on the correlation between degree of reduction in plasma or cellular Gb3 levels and clinical benefit.
Negative_regulation (reduction) of Gb3 in plasma
10) Confidence 0.09 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.56 Pain Relevance 0
In urine sediment, the magnitude of reduction in Gb3 was about 50% (Schiffmann et al 2001).
Negative_regulation (reduction) of Gb3 in urine
11) Confidence 0.09 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.87 Pain Relevance 0.61
In the male patients, plasma Gb3 level declined by 43% after 6 months (p < 0.001).
Negative_regulation (declined) of Gb3 in plasma
12) Confidence 0.09 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.65 Pain Relevance 0
Compounds with chaperone activity would reduce Gb3 storage in the lysosome, and thus, Gb3 staining in cells should become distinctively diminished after treatment with active compounds.
Negative_regulation (diminished) of Gb3
13) Confidence 0.06 Published 2010 Journal Current Chemical Genomics Section Body Doc Link PMC2995157 Disease Relevance 0 Pain Relevance 0
Depending on the potency and selectivity of active compounds identified in the above processes, a probe can be defined if the potencies (IC50/EC50) of the identified agents are less than 1 ┬ÁM, and if they selectively act on GLA and effectively reduce Gb3 accumulation in patient cells.
Negative_regulation (reduce) of Gb3 associated with potency
14) Confidence 0.06 Published 2010 Journal Current Chemical Genomics Section Body Doc Link PMC2995157 Disease Relevance 0.15 Pain Relevance 0.09

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