| Host: |
Rabbit |
| Applications: |
WB/IHC-P/IF/ICC/ELISA |
| Reactivity: |
Human/Mouse/Rat |
| Note: |
STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Clonality: |
Polyclonal |
| Conjugation: |
Unconjugated |
| Isotype: |
IgG |
| Formulation: |
PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. |
| Purification: |
Affinity purification |
| Concentration: |
Lot specific |
| Dilution Range: |
WB:1:1000-1:2000IHC-P:1:50-1:200IF/ICC:1:50-1:200ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Storage Instruction: |
Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: |
SPG7 |
| Gene ID: |
6687 |
| Uniprot ID: |
SPG7_HUMAN |
| Immunogen Region: |
1-250 |
| Specificity: |
Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1). |
| Immunogen Sequence: |
MAVLLLLLRALRRGPGPGPR PLWGPGPAWSPGFPARPGRG RPYMASRPPGDLAEAGGRAL QSLQLRLLTPTFEGINGLLL KQHLVQNPVRLWQLLGGTFY FNTSRLKQKNKEKDKSKGKA PEEDEEERRRRERDDQMYRE RLRTLLVIAVVMSLLNALST SGGSISWNDFVHEMLAKGEV QRVQVVPESDVVEVYLHPGA VVFGRPRLALMYRMQVANID KFEEKLRAAEDELNIEAKD |
| Tissue Specificity | Ubiquitous. |
| 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
12 months for antibodies. 6 months for ELISA Kits. Please see website T&Cs for further guidance
