" /> Mitochondrial Inner Membrane m-AAA Protease Component Paraplegin - CISMeF





Preferred Label : Mitochondrial Inner Membrane m-AAA Protease Component Paraplegin;

NCIt synonyms : SPG7; Spastic Paraplegia 7 Protein; Cell Matrix Adhesion Regulator; EC 3.4.24.-; Paraplegin;

NCIt definition : Mitochondrial inner membrane m-AAA protease component paraplegin (795 aa, 88 kDa) is encoded by the human SPG7 gene. This protein is involved in both proteolysis and the formation and regulation of the mitochondrial permeability transition pore.;

NCIt note : Mitochondrial inner membrane m-AAA protease component paraplegin is a component of both the m-AAA protease complex, which is composed of heterohexamers of AFG3L2 and SPG7 and the mitochondrial permeability transition pore complex (mPTPC), which includes at least SPG7, VDAC1 and PPIF. (UniProt);

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07/05/2025


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