CIDeRplusGeneral Information:
| Id: | 1,752 |
| Diseases: |
Amyotrophic lateral sclerosis
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| Mus musculus | |
| SOD1G93A transgenic mouse, transfected with human SOD1G93A | |
| BTO:0001279 spinal cord | |
| article | |
| Reference: | Martin LJ et al.(2007) Motor neuron degeneration in amyotrophic lateral sclerosis mutant superoxide dismutase-1 transgenic mice: mechanisms of mitochondriopathy and cell death. J. Comp. Neurol. 500: 20-46 [PMID: 17099894] |
Interaction Information:
| Comment | The structure of degenerating MNs (motor neurons) in G93A-mSOD1 mice does not resemble apoptosis but rather slow necrosis. It is characterized by somal and mitochondrial swelling and formation of DNA single-strand breaks prior to double-strand breaks occurring in nuclear and mitochondrial DNA. |
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Formal Description Interaction-ID: 13136 |
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| Comment | The structure of degenerating MNs (motor neurons) in G93A-mSOD1 mice does not resemble apoptosis but rather slow necrosis. It is characterized by somal and mitochondrial swelling and formation of DNA single-strand breaks prior to double-strand breaks occurring in nuclear and mitochondrial DNA. |
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Formal Description Interaction-ID: 13160 |
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| Comment | MN (motor neuron) death in G93A-mSOD1 mice is independent of activation of caspases-1, -3, and -8 or AIF within MNs. |
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Formal Description Interaction-ID: 13161 |
gene/protein NOT affects_activity of phenotype |
| Drugbank entries | Show/Hide entries for CASP1 |
| Comment | MN (motor neuron) death in G93A-mSOD1 mice is independent of activation of caspases-1, -3, and -8 or AIF within MNs. |
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Formal Description Interaction-ID: 13162 |
gene/protein NOT affects_activity of phenotype |
| Drugbank entries | Show/Hide entries for CASP3 |
| Comment | MN (motor neuron) death in G93A-mSOD1 mice is independent of activation of caspases-1, -3, and -8 or AIF within MNs. |
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Formal Description Interaction-ID: 13163 |
gene/protein NOT affects_activity of phenotype |
| Comment | MNs (motor neurons) in G93A-mSOD1 mice have impaired Na,K-ATPase. |
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Formal Description Interaction-ID: 13166 |
gene/protein mutant decreases_activity of complex/PPI Sodium:potassium-exchanging ATPase complex |
| Comment | MN (motor neuron) death in G93A-mSOD1 mice is independent of activation of caspases-1, -3, and -8 or AIF within MNs. |
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Formal Description Interaction-ID: 13169 |
gene/protein NOT affects_activity of phenotype |
| Drugbank entries | Show/Hide entries for AIFM1 |
| Comment | Complex IV activity (measured by Cox-I protein) in lumbar spinal cord ventral horn samples was decreased in symptomatic mSOD1 mice, which corresponded to a progressive, severe aggregation of Cox-I protein. |
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Formal Description Interaction-ID: 13170 |
gene/protein mutant decreases_activity of complex/PPI Mitochondrial respiratory chain complex IV |
| Comment | The structure of degenerating MNs (motor neurons) in G93A-mSOD1 mice does not resemble apoptosis but rather slow necrosis. It is characterized by somal and mitochondrial swelling and formation of DNA single-strand breaks prior to double-strand breaks occurring in nuclear and mitochondrial DNA. |
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Formal Description Interaction-ID: 13176 |
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| Comment | G93A-mSOD1 mouse MNs (motor neurons) accumulate mitochondria and generate increased levels of ROS. |
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Formal Description Interaction-ID: 13177 |
gene/protein mutant increases_quantity of drug/chemical compound Reactive oxygen species |
| Comment | Intracellular Ca2+ was elevated significantly in mSOD1 mouse MNs (motor neurons) compared with age-matched wtSOD1 mouse MNs. |
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Formal Description Interaction-ID: 13178 |
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| Comment | NO production was elevated significantly in mSOD1 mouse MNs (motor neurons) compared with wtSOD1 mouse MNs. |
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Formal Description Interaction-ID: 13179 |
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| Comment | H2DCFDA (used to track ONOO-) fluorescence signal was significantly higher in mSOD1 mouse MNs (motor neurons) compared with wtSOD1 mouse MNs. |
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Formal Description Interaction-ID: 13180 |
gene/protein mutant increases_quantity of drug/chemical compound |
| Comment | The nitration of Cox-I in mitochondrial membrane-enriched fractions of lumbar spinal cord ventral horn was greater in mSOD1 mice compared with age-matched wtSOD1 mice confirming the presence of ONOO- at mitochondria. |
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Formal Description Interaction-ID: 13181 |
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| Comment | MNs (motor neurons) in presymptomatic mSOD1 mice had strong immunolabeling for iNOS. Immunogold-EM revealed that iNOS was localized to mitochondria and to the cytosol in mSOD1 mouse MNs at presymptomatic stages of disease. |
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Formal Description Interaction-ID: 13182 |
gene/protein mutant increases_quantity of gene/protein |
| Drugbank entries | Show/Hide entries for NOS2 |
| Comment | iNOS was localized to mitochondria and to the cytosol in mSOD1 mouse MNs at presymptomatic stages of disease. |
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Formal Description Interaction-ID: 13188 |
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| Drugbank entries | Show/Hide entries for NOS2 |
| Comment | iNOS was localized to mitochondria and to the cytosol in mSOD1 mouse MNs at presymptomatic stages of disease. |
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Formal Description Interaction-ID: 13189 |
gene/protein is localized in cellular component |
| Drugbank entries | Show/Hide entries for NOS2 |
| Comment | iNOS gene deletion extends significantly the life span of G93A-mSOD1 mice. |
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Formal Description Interaction-ID: 13190 |
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| Drugbank entries | Show/Hide entries for NOS2 |
| Comment | H2DCFDA (used to track ONOO-) fluorescence signal was significantly higher in mSOD1 mouse MNs (motor neurons) compared with wtSOD1 mouse MNs. |
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Formal Description Interaction-ID: 13625 |
gene/protein mutant increases_activity of process |