| Host: | Rabbit |
| Applications: | WB/IHC-P/IP/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 0.05% Proclin300, 50% Glycerol, pH 7.3. |
| Purification: | Affinity purification |
| Concentration: | Lot specific |
| Dilution Range: | WB:1:500-1:1000 IHC-P:1:50-1:200 IP:0.5 Mu g-4 Mu g antibody for 200 Mu g-400 Mu g extracts of whole cells ELISA: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: | AFG3L2 |
| Gene ID: | 10939 |
| Uniprot ID: | AFG32_HUMAN |
| Immunogen Region: | 538-797 |
| Specificity: | Recombinant fusion protein containing a sequence corresponding to amino acids 538-797 of human AFG3L2 (NP_006787.2). |
| Immunogen Sequence: | DSINQKHFEQAIERVIGGLE KKTQVLQPEEKKTVAYHEAG HAVAGWYLEHADPLLKVSII PRGKGLGYAQYLPKEQYLYT KEQLLDRMCMTLGGRVSEEI FFGRITTGAQDDLRKVTQSA YAQIVQFGMNEKVGQISFDL PRQGDMVLEKPYSEATARLI DDEVRILINDAYKRTVALLT EKKADVEKVALLLLEKEVLD KNDMVELLGPRPFAEKSTYE EFVEGTGSLDEDTSLPEGL |
| Tissue Specificity | Ubiquitous. Highly expressed in the cerebellar Purkinje cells. |
| Post Translational Modifications | Upon import into the mitochondrion, the N-terminal transit peptide is cleaved to generate an intermediate form which undergoes autocatalytic proteolytic processing 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. AFG3L2 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 carries out quality control in the inner membrane of the mitochondria by mediating degradation of mistranslated or misfolded polypeptides. The m-AAA protease complex also promotes the processing and maturation of mitochondrial proteins, such as MRPL32/bL32m, PINK1 and SP7. 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. Required for SPG7 maturation into its active mature form after SPG7 cleavage by mitochondrial-processing peptidase (MPP). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP). 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. Promotes the proteolytic degradation of GHITM upon hyperpolarization of mitochondria: progressive GHITM degradation leads to respiratory complex I degradation and broad reshaping of the mitochondrial proteome by AFG3L2. Also acts as a regulator of mitochondrial glutathione homeostasis by mediating cleavage and degradation of SLC25A39. SLC25A39 cleavage is prevented when SLC25A39 binds iron-sulfur. Involved in the regulation of OMA1-dependent processing of OPA1. May act by mediating processing of OMA1 precursor, participating in OMA1 maturation. |
| Protein Name | Mitochondrial Inner Membrane M-Aaa Protease Component Afg3l2 Afg3-Like Protein 2 Paraplegin-Like Protein |
| Database Links | Reactome: R-HSA-8949664 Reactome: R-HSA-9837999 |
| Cellular Localisation | Mitochondrion Inner Membrane Multi-Pass Membrane Protein |
| Alternative Antibody Names | Anti-Mitochondrial Inner Membrane M-Aaa Protease Component Afg3l2 antibody Anti-Afg3-Like Protein 2 antibody Anti-Paraplegin-Like Protein antibody Anti-AFG3L2 antibody |
Information sourced from Uniprot.org