INT98239

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Context Info
Confidence 0.43
First Reported 2001
Last Reported 2010
Negated 0
Speculated 1
Reported most in Body
Documents 13
Total Number 14
Disease Relevance 7.84
Pain Relevance 0.60

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

Golgi apparatus (Htt) endoplasmic reticulum (Htt) mitochondrion organization (Htt)
embryo development (Htt) protein complex (Htt) cytoplasm (Htt)
Anatomy Link Frequency
testes 2
brain 2
neuronal 1
muscles 1
striatum 1
Htt (Mus musculus)
Pain Link Frequency Relevance Heat
Glutamate 72 94.80 High High
nMDA receptor 39 81.92 Quite High
depression 6 78.32 Quite High
corticosteroid 4 76.04 Quite High
agonist 10 75.44 Quite High
Pyramidal cell 24 73.52 Quite High
Central nervous system 19 69.20 Quite High
midbrain 1 66.56 Quite High
Thalamus 8 64.56 Quite High
Hippocampus 11 59.00 Quite High
Disease Link Frequency Relevance Heat
Disease 610 100.00 Very High Very High Very High
Toxicity 70 99.20 Very High Very High Very High
Targeted Disruption 224 95.20 Very High Very High Very High
Death 51 94.40 High High
Neurodegenerative Disease 62 94.00 High High
Weight Loss 62 91.24 High High
Disease Progression 26 90.00 High High
Frailty 61 85.84 High High
Hypotension 3 82.56 Quite High
Cognitive Disorder 13 80.32 Quite High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
Neurons lacking huntingtin differentially colonize brain and survive in chimeric mice.
Negative_regulation (lacking) of huntingtin in brain
1) Confidence 0.43 Published 2001 Journal J. Neurosci. Section Title Doc Link 11567051 Disease Relevance 0.06 Pain Relevance 0.07
We had attributed these widespread pathologies in the R6/2 mouse brain to the expression of a small N-terminal fragment of Htt and therefore were surprised to find that it was also a feature of the HdhQ150/Q150 mice.
Negative_regulation (feature) of HdhQ150 in brain
2) Confidence 0.43 Published 2009 Journal PLoS ONE Section Body Doc Link PMC2778556 Disease Relevance 0.71 Pain Relevance 0
Therefore, we have demonstrated that the peripheral pathology documented in the R6/2 mice is not the result of the aberrant expression of a small N-terminal fragment of mutant Htt as it is also present in a genetically precise HD mouse model in which an expanded CAG repeat has been inserted into the mouse Hdh gene.
Negative_regulation (precise) of HD associated with disease
3) Confidence 0.37 Published 2009 Journal PLoS ONE Section Body Doc Link PMC2778556 Disease Relevance 0.51 Pain Relevance 0
We have shown that the testes are reduced in size in both R6/2 and HdhQ150/Q150 mice, also reported in the YAC128 HD mouse model [55], and testosterone levels are reduced in both R6/2 mice [56] and male HD patients [54], [57].
Negative_regulation (reduced) of HD in testes associated with disease
4) Confidence 0.37 Published 2009 Journal PLoS ONE Section Body Doc Link PMC2778556 Disease Relevance 1.21 Pain Relevance 0.04
The improvement of multiple behavioural parameters throughout the rilmenidine treatment period was also associated with a decrease in the levels of soluble mutant huntingtin.
Negative_regulation (decrease) of mutant huntingtin
5) Confidence 0.26 Published 2010 Journal Human Molecular Genetics Section Body Doc Link PMC2865373 Disease Relevance 0.46 Pain Relevance 0.03
The marked improvement in behavioural outcome associated with the decrease in soluble mutant huntingtin seen in these mice is consistent with this view.


Negative_regulation (decrease) of mutant huntingtin
6) Confidence 0.26 Published 2010 Journal Human Molecular Genetics Section Body Doc Link PMC2865373 Disease Relevance 0.21 Pain Relevance 0.03
One of the outcomes of excitatory activity in the corticostriatal pathway is the concomitant release of BDNF (brain-derived neurotrophic factor), a trophic factor essential for postsynaptic neuronal support that is reduced in HD (Zuccato and Cattaneo, 2007).
Negative_regulation (reduced) of HD in neuronal
7) Confidence 0.23 Published 2010 Journal ASN NEURO Section Body Doc Link PMC2850512 Disease Relevance 0.14 Pain Relevance 0.12
Inhibition of complex II with 3-nitropropionic acid became a useful model of HD because it produced lesions in the striatum that were similar to the cell loss in HD (Beal et al., 1993; Damiano et al., 2009).
Negative_regulation (loss) of HD in striatum
8) Confidence 0.23 Published 2010 Journal ASN NEURO Section Body Doc Link PMC2850512 Disease Relevance 0.14 Pain Relevance 0.22
Rilmenidine decreases levels of mutant huntingtin
Negative_regulation (decreases) of mutant huntingtin
9) Confidence 0.19 Published 2010 Journal Human Molecular Genetics Section Body Doc Link PMC2865373 Disease Relevance 1.14 Pain Relevance 0
We have shown that the testes are reduced in size in both R6/2 and HdhQ150/Q150 mice, also reported in the YAC128 HD mouse model [55], and testosterone levels are reduced in both R6/2 mice [56] and male HD patients [54], [57].
Negative_regulation (reduced) of HdhQ150 in testes associated with disease
10) Confidence 0.19 Published 2009 Journal PLoS ONE Section Body Doc Link PMC2778556 Disease Relevance 1.29 Pain Relevance 0.04
Cell size is decreased in HD mice (Levine et al., 1999; Klapstein et al., 2001) and K+ conductances are reduced (Ariano et al., 2005).
Negative_regulation (decreased) of HD
11) Confidence 0.16 Published 2010 Journal ASN NEURO Section Body Doc Link PMC2850512 Disease Relevance 0.08 Pain Relevance 0.06
To test whether overexpression of PSA causes a similar suppression of huntingtin aggregates in mouse tissues in vivo, we electroporated GFP-tagged huntingtin exon 1 constructs bearing different polyQ lengths into the tibialis anterior muscles of adult mice.
Spec (whether) Negative_regulation (suppression) of huntingtin in muscles
12) Confidence 0.07 Published 2010 Journal Human Molecular Genetics Section Body Doc Link PMC2972693 Disease Relevance 0.15 Pain Relevance 0
Furthermore, activation of this autophagic process (e.g. by rapamycin) enhances the removal of the aggregate-prone proteins such as mutant huntingtin and attenuates its toxicity in cell and animal models (2).
Negative_regulation (removal) of huntingtin associated with toxicity
13) Confidence 0.06 Published 2010 Journal Human Molecular Genetics Section Body Doc Link PMC2972693 Disease Relevance 0.60 Pain Relevance 0
Inhibition of autophagy by chemical regulators, such as bafilomycin A1 and 3-methyladenine, decreases the clearance of aggregate-prone proteins such as mutant huntingtin (that causes Huntington's disease) or mutant forms of alpha-synuclein that cause familial Parkinson's disease.
Negative_regulation (decreases) of mutant huntingtin associated with disease
14) Confidence 0.05 Published 2008 Journal Biochim Biophys Acta Section Body Doc Link PMC2597715 Disease Relevance 1.14 Pain Relevance 0

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