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Literature summary extracted from
- Loss of the Drosophila m-AAA mitochondrial protease paraplegin results in mitochondrial dysfunction, shortened lifespan, and neuronal and muscular degeneration article (2018), Cell Death Dis., 9, 304 .
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 3.4.24.B18 | Drosophila melanogaster | Q9W4W8 | - |
- |
Source Tissue
| EC Number | Source Tissue | Comment | Organism | Textmining |
|---|
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 3.4.24.B18 | paraplegin | - |
Drosophila melanogaster |
| 3.4.24.B18 | SPG7 | - |
Drosophila melanogaster |
General Information
| EC Number | General Information | Comment | Organism |
|---|---|---|---|
| 3.4.24.B18 | physiological function | SPG7 mutants exhibit shortened lifespan, progressive locomotor defects, sensitivity to chemical and environmental stress, and muscular and neuronal degeneration. The neurodegenerative phenotype of SPG7 mutants initiates at the synaptic terminal. A variety of mitochondrial defects are observed in the mutants, including altered axonal transport of mitochondria, accumulation of electron-dense material in the matrix of flight muscle mitochondria, reduced activities of respiratory chain complexes I and II, and severely swollen and dysmorphic mitochondria in the synaptic terminals of photoreceptors | Drosophila melanogaster |
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