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Literature summary for 3.4.24.B18 extracted from

  • Hurst, S.; Baggett, A.; Csordas, G.; Sheu, S.S.
    SPG7 targets the m-AAA protease complex to process MCU for uniporter assembly, Ca2+ influx, and regulation of mitochondrial permeability transition pore opening (2019), J. Biol. Chem., 294, 10807-10818 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.24.B18

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
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 Homo sapiens 5739
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Organism

Organism UniProt Comment Textmining
Homo sapiens Q9UQ90 and Q9Y4W6 Q9UQ90 i.e. subunit SPG7, Q9Y4W6 i.e. subunit Afg3l2
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Synonyms

Synonyms Comment Organism
paraplegin
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 Homo sapiens
SPG7
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 Homo sapiens

General Information

General Information Comment Organism
physiological function loss of the SPG7 gene increased resistance to Ca2+-induced mitochondrial permeability transition pore opening. This occurs independently of cyclophilin D (cyclosporine A insensitive) but rather it is through decreased mitochondrial Ca2+ concentrations and subsequent adaptations mediated by impaired formation of functional mitochondrial Ca2+ uniporter complexes. SPG7 directs the m-AAA complex to favor association with the mitochondrial Ca2+ uniporter, and mitochondrial Ca2+ uniporter processing regulates higher order mitochondrial Ca2+ uniporter-complex formation Homo sapiens