INT36179

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Context Info
Confidence 0.44
First Reported 1988
Last Reported 2010
Negated 1
Speculated 2
Reported most in Body
Documents 11
Total Number 13
Disease Relevance 6.56
Pain Relevance 1.12

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

small molecule metabolic process (GLA) extracellular region (GLA) Golgi apparatus (GLA)
lysosome (GLA) cytoplasm (GLA)
Anatomy Link Frequency
endothelial cells 2
GLA (Homo sapiens)
Pain Link Frequency Relevance Heat
Pain 82 100.00 Very High Very High Very High
Potency 5 96.14 Very High Very High Very High
tolerance 9 95.24 Very High Very High Very High
visual analogue scale 1 92.80 High High
Bioavailability 1 85.28 High High
cva 30 79.88 Quite High
headache 5 75.04 Quite High
carpal tunnel syndrome 1 75.00 Quite High
aspirin 10 73.92 Quite High
Calcium channel 6 71.24 Quite High
Disease Link Frequency Relevance Heat
Pain 105 100.00 Very High Very High Very High
Disease 205 99.74 Very High Very High Very High
Renal Disease 177 97.36 Very High Very High Very High
Disease Progression 12 97.24 Very High Very High Very High
Hypertension 10 97.24 Very High Very High Very High
Coronary Heart Disease 52 95.12 Very High Very High Very High
Fabry Disease 391 93.08 High High
Angiokeratoma 14 90.72 High High
Disorders Of Creatine Metabolism 7 88.72 High High
Renal Insufficiency 15 83.44 Quite High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
The defects in the GLA gene encoding ?
Regulation (defects) of GLA
1) Confidence 0.44 Published 2010 Journal Orphanet J Rare Dis Section Body Doc Link PMC3009617 Disease Relevance 0.54 Pain Relevance 0
Calcitonin therapy did not affect serum calcium, alkaline phosphatase, bone gla-protein, or the urinary excretion of calcium and hydroxyproline.
Neg (not) Regulation (affect) of gla
2) Confidence 0.44 Published 1988 Journal Bone Section Abstract Doc Link 3288258 Disease Relevance 0.91 Pain Relevance 0.31
Fabry's disease, a disorder affecting the gene for the lysosomal enzyme alpha-galactosidase A (alpha-GAL A), can cause accumulation of globotriaosylceramide (GL-3) in the vascular endothelial cells.
Regulation (affecting) of alpha-galactosidase A in endothelial cells associated with disease
3) Confidence 0.44 Published 2007 Journal J. Int. Med. Res. Section Abstract Doc Link 17697536 Disease Relevance 0.49 Pain Relevance 0.09
Fabry's disease, a disorder affecting the gene for the lysosomal enzyme alpha-galactosidase A (alpha-GAL A), can cause accumulation of globotriaosylceramide (GL-3) in the vascular endothelial cells.
Regulation (affecting) of alpha-GAL A in endothelial cells associated with disease
4) Confidence 0.44 Published 2007 Journal J. Int. Med. Res. Section Abstract Doc Link 17697536 Disease Relevance 0.49 Pain Relevance 0.09
The first placebo-controlled clinical trial with agalsidase alfa has shown a beneficial effect on pain.
Regulation (controlled) of agalsidase alfa associated with pain
5) Confidence 0.42 Published 2007 Journal PLoS ONE Section Body Doc Link PMC1913555 Disease Relevance 1.00 Pain Relevance 0.17
In summary, the early studies reported mainly a beneficial effect on clinical outcome (pain, renal function) with agalsidase alfa, and reductions in stored GL-3 in the agalsidase beta studies.
Regulation (effect) of agalsidase alfa associated with pain
6) Confidence 0.42 Published 2007 Journal PLoS ONE Section Body Doc Link PMC1913555 Disease Relevance 0.76 Pain Relevance 0.14
Effects of agalsidase alfa and beta therapies on clinical parameters
Regulation (Effects) of agalsidase alfa
7) Confidence 0.37 Published 2007 Journal PLoS ONE Section Body Doc Link PMC1913555 Disease Relevance 0.19 Pain Relevance 0.11
The Hi-Ob scale showed significant efficacy of ALA/GLA in improving symptoms and functional impairment, while in the multivitamin group the improvement was significant, but less marked than in the ALA/GLA group.
Regulation (efficacy) of GLA
8) Confidence 0.26 Published 2009 Journal Eur Rev Med Pharmacol Sci Section Abstract Doc Link 19499849 Disease Relevance 0.38 Pain Relevance 0.07
After these initial cases, other associated symptoms were described and eventually mutations in the alpha-galactosidase A (AGAL) gene were found to be responsible for the disease (Brady et al 1967), which is an X-linked disorder of glycosphingolipid metabolism.
Regulation (responsible) of alpha-galactosidase associated with disease
9) Confidence 0.26 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.49 Pain Relevance 0
Despite differences in glycosylation that could affect tissue delivery and uptake (Lee et al 2003), agalsidase-alfa and agalsidase-beta appear have identical immunogenicity and specific activity (Lee et al 2003; Linthorst et al 2004).
Regulation (affect) of agalsidase
10) Confidence 0.26 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.14 Pain Relevance 0.05
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.
Regulation (act) of GLA associated with potency
11) Confidence 0.21 Published 2010 Journal Current Chemical Genomics Section Body Doc Link PMC2995157 Disease Relevance 0.14 Pain Relevance 0.09
Residual alpha-galactosidase A activity as well as other genetic factors may affect the phenotype.
Spec (may) Regulation (affect) of alpha-galactosidase
12) Confidence 0.15 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.87 Pain Relevance 0
We report the results of an open-label phase 2 study that was undertaken to evaluate whether ethnic differences exist that would affect agalsidase beta (Fabrazyme) treatment of Fabry patients in the Japanese population, relative to safety and efficacy.
Spec (whether) Regulation (affect) of agalsidase beta
13) Confidence 0.04 Published 2005 Journal J. Inherit. Metab. Dis. Section Abstract Doc Link 15902561 Disease Relevance 0.16 Pain Relevance 0

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