INT167153

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Context Info
Confidence 0.49
First Reported 2007
Last Reported 2010
Negated 0
Speculated 0
Reported most in Body
Documents 10
Total Number 20
Disease Relevance 10.45
Pain Relevance 1.71

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 5
urine 3
cardiomyocytes 2
endothelial cells 2
epithelial cells 1
A4GALT (Homo sapiens)
Pain Link Frequency Relevance Heat
Pain 385 99.60 Very High Very High Very High
Neuropathic pain 64 98.04 Very High Very High Very High
imagery 274 96.08 Very High Very High Very High
fibrosis 107 78.88 Quite High
cva 72 60.64 Quite High
Pain score 16 45.20 Quite Low
Mcgill questionnaire 11 42.72 Quite Low
Antihistamine 27 32.24 Quite Low
corticosteroid 16 30.96 Quite Low
palliative 17 5.52 Low Low
Disease Link Frequency Relevance Heat
Pain 445 99.60 Very High Very High Very High
Fabry Disease 2527 99.46 Very High Very High Very High
Lysosome Storage Disease 74 99.20 Very High Very High Very High
Colon Cancer 21 98.84 Very High Very High Very High
Cancer 115 98.28 Very High Very High Very High
Neuropathic Pain 97 98.04 Very High Very High Very High
Disease 696 98.00 Very High Very High Very High
Congenital Anomalies 203 95.24 Very High Very High Very High
Hypertrophy 34 84.64 Quite High
Hypohidrosis 33 84.00 Quite High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
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.
Gene_expression (levels) of Gb3 in plasma associated with pain
1) Confidence 0.49 Published 2010 Journal Curr. Med. Chem. Section Abstract Doc Link 20345350 Disease Relevance 0.91 Pain Relevance 0.14
The authors concluded that high dose treatment may be more effective in reducing Gb3-levels, however, following treatment failure seven patients in this cohort received increased dosages of Agalsidase during the study, and five of them developed worsening of ischemic lesions in the CNS.
Gene_expression (reducing) of Gb3
2) Confidence 0.33 Published 2009 Journal Orphanet J Rare Dis Section Body Doc Link PMC2768700 Disease Relevance 0.06 Pain Relevance 0
All of the silenced cells had reduced viability, significant accumulation of intracellular Gb3, and a modest but significant increase in membranous Gb3 (CD77) expression compared to non-silenced cells.
Gene_expression (expression) of Gb3
3) Confidence 0.26 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.41 Pain Relevance 0
When silenced HK2 cells were reconstituted with agalsidase alfa, they decreased their membranous CD77 expression to levels indistinguishable from those of non-silenced cells.
Gene_expression (expression) of CD77
4) Confidence 0.26 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.41 Pain Relevance 0
All of the silenced cells had reduced viability, significant accumulation of intracellular Gb3, and a modest but significant increase in membranous Gb3 (CD77) expression compared to non-silenced cells.
Gene_expression (expression) of CD77
5) Confidence 0.26 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.41 Pain Relevance 0
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.
Gene_expression (levels) of Gb3 in plasma associated with neuropathic pain
6) Confidence 0.22 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.59 Pain Relevance 0.19
In classically affected Fabry patients, renal lesions result from Gb3 deposition in the glomerular endothelial, mesangial, intersticial cells and in podocytes (Figures 3 and 4), which are terminally-differenciated epithelial cells that accumulate numerous myelin-like inclusions in their lysosomes (Figure 5).
Gene_expression (deposition) of Gb3 in epithelial cells
7) Confidence 0.22 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.41 Pain Relevance 0
No clearance of Gb3 was observed in the cardiomyocytes during the trial [359].
Gene_expression (clearance) of Gb3 in cardiomyocytes
8) Confidence 0.22 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0 Pain Relevance 0
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.
Gene_expression (levels) of Gb3 in plasma associated with pain and fabry disease
9) Confidence 0.22 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.77 Pain Relevance 0.41
These data suggest that membranous CD77 levels may mirror Gb3 tissue load and that CD77 expression levels may be used to monitor the efficacy of ERT [390].


Gene_expression (expression) of CD77
10) Confidence 0.22 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.47 Pain Relevance 0
Mean urine Gb3 levels were reduced to normal range.
Gene_expression (levels) of Gb3 in urine
11) Confidence 0.20 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.76 Pain Relevance 0.46
Cardiomyocyte Gb3 clearance which was the primary efficacy endpoint did not reach statistical significance [326].
Gene_expression (clearance) of Gb3 in Cardiomyocyte
12) Confidence 0.20 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.78 Pain Relevance 0.10
Before treatment, results of skin biopsies from 12 male patients showed moderate or severe Gb3 accumulation in superficial dermal capillary endothelial cells; with treatment, these cells were completely cleared of Gb3 in week-24 biopsies from all 12 male patients and in all available week-48 biopsies.
Gene_expression (cleared) of Gb3 in endothelial cells
13) Confidence 0.20 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.29 Pain Relevance 0.07
In the latter study, all patients had normal plasma Gb3 levels and only a single patient had visible storage material in the superficial dermal vascular endothelial cells.
Gene_expression (levels) of Gb3 in endothelial cells
14) Confidence 0.15 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.56 Pain Relevance 0
No significant difference was found in reduction of left ventricular mass (the primary endpoint) or changes in other disease parameters (ie, glomerular filtration rate, pain, anti-agalsidase antibodies, and plasma and urine Gb3 levels), after 12 and 24 months of treatment with either enzyme formulation.
Gene_expression (levels) of Gb3 in urine associated with pain and disease
15) Confidence 0.15 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.72 Pain Relevance 0.17
In a study involving a subgroup of patients (n = 8) with a persistent IgG antibody response, mean urine Gb3 levels were observed to rise, reaching mean baseline (Linthorst et al 2004).
Gene_expression (levels) of Gb3 in urine
16) Confidence 0.13 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.17 Pain Relevance 0
The source of Gb3 found in plasma is not known, and may reflect a surplus from the cellular turnover of membranes from a variety of organs and newly synthesized substrate in transit.
Gene_expression (source) of Gb3 in plasma
17) Confidence 0.12 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.55 Pain Relevance 0
This approach involves the use of a glucosylceramide synthase inhibitor, which would slow the rate of Gb3 synthesis, and thus decrease lysosomal storage.
Gene_expression (synthesis) of Gb3 associated with lysosome storage disease
18) Confidence 0.11 Published 2010 Journal Current Chemical Genomics Section Body Doc Link PMC2995157 Disease Relevance 0.51 Pain Relevance 0
The finding that Gb3 is predominantly found in cholesterol-rich microdomains (CRM) of tumor, but not of normal cells might provide an explanation for the tumor-specific Hsp70 membrane expression.
Gene_expression (found) of Gb3 associated with cancer
19) Confidence 0.07 Published 2009 Journal J Transl Med Section Body Doc Link PMC2714296 Disease Relevance 0.94 Pain Relevance 0
No significant difference was found in reduction of left ventricular mass (the primary endpoint) or changes in other disease parameters (ie, glomerular filtration rate, pain, anti-agalsidase antibodies, and plasma and urine Gb3 levels), after 12 and 24 months of treatment with either enzyme formulation.
Gene_expression (levels) of Gb3 in plasma associated with pain and disease
20) Confidence 0.05 Published 2007 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2721310 Disease Relevance 0.72 Pain Relevance 0.17

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