INT216103

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Context Info
Confidence 0.46
First Reported 2007
Last Reported 2010
Negated 0
Speculated 0
Reported most in Body
Documents 15
Total Number 17
Disease Relevance 5.92
Pain Relevance 1.21

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

plasma membrane (Dmd) cytoskeleton (Dmd) nucleus (Dmd)
cytoplasm (Dmd)
Anatomy Link Frequency
cardiac muscle 3
Muscles 3
muscle cells 1
hindlimb 1
synapses 1
Dmd (Mus musculus)
Pain Link Frequency Relevance Heat
Neurotransmitter 88 98.52 Very High Very High Very High
Hippocampus 48 97.40 Very High Very High Very High
tetrodotoxin 64 95.98 Very High Very High Very High
Central nervous system 32 88.40 High High
Glutamate 12 83.00 Quite High
Potency 15 62.12 Quite High
Glutamate receptor 24 51.00 Quite High
fibrosis 109 39.00 Quite Low
Calcium channel 6 24.12 Low Low
GABAergic 32 18.08 Low Low
Disease Link Frequency Relevance Heat
Muscular Dystrophy 725 97.66 Very High Very High Very High
Cardiomyopathy 189 96.32 Very High Very High Very High
Congenital Anomalies 67 95.44 Very High Very High Very High
Cognitive Disorder 48 92.00 High High
Death 80 88.56 High High
Helminth Infection 28 87.80 High High
Hypoxia 3 85.76 High High
Lifespan 14 85.64 High High
Disease 41 79.12 Quite High
Apoptosis 19 76.64 Quite High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
Taking into account that currents in cell-attached patches were completely blocked by TTX, we confirm that Nav1.4 is the primary isoform expressed in the sarcolemma of both dystrophic and control fibers and that the up-regulated activity of Nav1.4 channels is primarily responsible for the increase in Na+ influx in dystrophin-deficient muscle cells, significantly affecting their survival.
Positive_regulation (increase) of dystrophin in muscle cells associated with tetrodotoxin
1) Confidence 0.46 Published 2008 Journal The Journal of General Physiology Section Body Doc Link PMC2483333 Disease Relevance 0.39 Pain Relevance 0.43
While CSA was increased in the mdx 11L EDL, tetanic force production was unchanged (Table 2).
Positive_regulation (increased) of mdx
2) Confidence 0.44 Published 2010 Journal PLoS ONE Section Body Doc Link PMC2820101 Disease Relevance 0.06 Pain Relevance 0
Thus, it has been shown that the large dystrophin isoform DLP2 at the glutamatergic NMJ and the smaller Dp186 isoform at cholinergic central synapses, both postsynaptically localized, are required for wild-type levels of presynaptic neurotransmitter release.
Positive_regulation (required) of dystrophin in synapses associated with neurotransmitter
3) Confidence 0.44 Published 2009 Journal Mol Neurobiol Section Body Doc Link PMC2840664 Disease Relevance 0.15 Pain Relevance 0.14
In summary, the requirements for dystrophin, dystroglycan, POMT, and sarcoglycan in maintaining muscle integrity in flies illustrate the usefulness of this invertebrate as a model system for the study of muscular dystrophies.


Positive_regulation (requirements) of dystrophin in muscle associated with muscular dystrophy
4) Confidence 0.44 Published 2009 Journal Mol Neurobiol Section Body Doc Link PMC2840664 Disease Relevance 0.36 Pain Relevance 0
As can be seen in Fig. 1, Na+ influx increased by 20 ± 5.6% (P = 0.003) in mdx5cv muscles compared with that of control FDB muscles.
Positive_regulation (increased) of mdx5cv in muscles
5) Confidence 0.40 Published 2008 Journal The Journal of General Physiology Section Body Doc Link PMC2483333 Disease Relevance 0 Pain Relevance 0.21
10 mV toward more positive potentials for mdx5cv in comparison with those of control fibers (?
Positive_regulation (potentials) of mdx5cv
6) Confidence 0.40 Published 2008 Journal The Journal of General Physiology Section Body Doc Link PMC2483333 Disease Relevance 0 Pain Relevance 0
The following section discusses new mechanistic insights into requirement for dystrophin in receptor clustering and synaptic plasticity in the mammalian hippocampus and cerebellum gained, in part, through electrophysiological analyses.
Positive_regulation (requirement) of dystrophin in cerebellum associated with hippocampus
7) Confidence 0.39 Published 2009 Journal Mol Neurobiol Section Body Doc Link PMC2840664 Disease Relevance 0.23 Pain Relevance 0.20
The relatively mild phenotype and near normal life span of the mdx mouse has prompted the generation of double mutants such as the mdx/utrophin and the mdx/MyoD mice, both of which have a far more severe skeletal and cardiac muscle phenotype to model the development of cardiomyopathy.
Positive_regulation (generation) of mdx in cardiac muscle associated with cardiomyopathy
8) Confidence 0.29 Published 2010 Journal The Open Cardiovascular Medicine Journal Section Body Doc Link PMC3024556 Disease Relevance 1.43 Pain Relevance 0
The relatively mild phenotype and near normal life span of the mdx mouse has prompted the generation of double mutants such as the mdx/utrophin and the mdx/MyoD mice, both of which have a far more severe skeletal and cardiac muscle phenotype to model the development of cardiomyopathy.
Positive_regulation (/) of mdx in cardiac muscle associated with cardiomyopathy
9) Confidence 0.29 Published 2010 Journal The Open Cardiovascular Medicine Journal Section Body Doc Link PMC3024556 Disease Relevance 1.41 Pain Relevance 0
Western analysis showed that the highest level of dystrophin induction was achieved using 5 ?
Positive_regulation (induction) of dystrophin
10) Confidence 0.26 Published 2008 Journal BMC Biotechnol Section Body Doc Link PMC2362111 Disease Relevance 0 Pain Relevance 0
The relatively mild phenotype and near normal life span of the mdx mouse has prompted the generation of double mutants such as the mdx/utrophin and the mdx/MyoD mice, both of which have a far more severe skeletal and cardiac muscle phenotype to model the development of cardiomyopathy.
Positive_regulation (generation) of mdx in cardiac muscle associated with cardiomyopathy
11) Confidence 0.25 Published 2010 Journal The Open Cardiovascular Medicine Journal Section Body Doc Link PMC3024556 Disease Relevance 1.44 Pain Relevance 0
The ineptitude of 2'OMe ESO delivery without a carrier in the mdx mouse has led to omitting ESO alone in other studies examining dystrophin induction following local and systemic delivery [11,16].


Positive_regulation (induction) of dystrophin
12) Confidence 0.23 Published 2008 Journal BMC Biotechnol Section Body Doc Link PMC2362111 Disease Relevance 0 Pain Relevance 0
Muscles from polyplex-treated mdx mice were analyzed for nNOS expression as evidence for expression of functional dystrophin with an intact N-terminal binding domain.
Positive_regulation (evidence) of dystrophin in Muscles
13) Confidence 0.16 Published 2008 Journal BMC Biotechnol Section Body Doc Link PMC2362111 Disease Relevance 0 Pain Relevance 0.03
This process may underlie to some extent the high number of dystrophin-positive fibers observed in our 6 week (10 injection) trials.
Positive_regulation (underlie) of dystrophin
14) Confidence 0.16 Published 2008 Journal BMC Biotechnol Section Body Doc Link PMC2362111 Disease Relevance 0 Pain Relevance 0
The growing opinion that ESOs are on the path to becoming a viable therapeutic option for DMD is well supported by cell culture and animal data showing specific skipping of various targeted exons and resultant induction of nearly full-length dystrophin [4,6,7,9,11-16,49,50].
Positive_regulation (induction) of dystrophin associated with muscular dystrophy
15) Confidence 0.15 Published 2008 Journal BMC Biotechnol Section Body Doc Link PMC2362111 Disease Relevance 0.18 Pain Relevance 0
The following section discusses new mechanistic insights into requirement for dystrophin in receptor clustering and synaptic plasticity in the mammalian hippocampus and cerebellum gained, in part, through electrophysiological analyses.
Positive_regulation (requirement) of dystrophin in hippocampus associated with hippocampus
16) Confidence 0.13 Published 2009 Journal Mol Neurobiol Section Body Doc Link PMC2840664 Disease Relevance 0.23 Pain Relevance 0.20
Vector genome titers were measured by Taqman Q-PCR as previously described by our group. [22]

ILP delivery of rAAV6 and rAAV8 results in efficient micro-dystrophin in the mouse hindlimb

Positive_regulation (results) of micro-dystrophin in hindlimb
17) Confidence 0.08 Published 2007 Journal J Transl Med Section Body Doc Link PMC2082019 Disease Relevance 0.05 Pain Relevance 0

General Comments

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