| Post Translational Modifications | Upon import into the mitochondrion, the N-terminal transit peptide is cleaved by the mitochondrial-processing peptidase (MPP) to generate an intermediate form which undergoes a second proteolytic cleavage mediated by proteases AFG3L2 removing an additional N-terminal fragment to generate the proteolytically active mature form. |
| Function | Catalytic component of the m-AAA protease, a protease that plays a key role in proteostasis of inner mitochondrial membrane proteins, and which is essential for axonal and neuron development. SPG7 possesses both ATPase and protease activities: the ATPase activity is required to unfold substrates, threading them into the internal proteolytic cavity for hydrolysis into small peptide fragments. The m-AAA protease exerts a dual role in the mitochondrial inner membrane: it mediates the processing of specific regulatory proteins and ensures protein quality control by degrading misfolded polypeptides. Mediates protein maturation of the mitochondrial ribosomal subunit MRPL32/bL32m by catalyzing the cleavage of the presequence of MRPL32/bL32m prior to assembly into the mitochondrial ribosome. Acts as a regulator of calcium in neurons by mediating degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU. Also regulates mitochondrial calcium by catalyzing degradation of MCU. Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function. |
| Protein Name | Mitochondrial Inner Membrane M-Aaa Protease Component ParapleginCell Matrix Adhesion RegulatorParapleginSpastic Paraplegia 7 Protein |
| Database Links | Reactome: R-HSA-8949664Reactome: R-HSA-9837999 |
| Cellular Localisation | Mitochondrion Inner MembraneMulti-Pass Membrane Protein |
| Alternative Antibody Names | Anti-Mitochondrial Inner Membrane M-Aaa Protease Component Paraplegin antibodyAnti-Cell Matrix Adhesion Regulator antibodyAnti-Paraplegin antibodyAnti-Spastic Paraplegia 7 Protein antibodyAnti-SPG7 antibodyAnti-CAR antibodyAnti-CMAR antibodyAnti-PGN antibody |
Information sourced from Uniprot.org
