SPG7 Antibody (Center) Blocking peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Primary Accession | Q9UQ90 |
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Clone Names | 90805109 |
Gene ID | 6687 |
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Other Names | Paraplegin, 3424-, Spastic paraplegia 7 protein, SPG7, CAR, CMAR, PGN |
Format | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C. |
Precautions | This product is for research use only. Not for use in diagnostic or therapeutic procedures. |
Name | SPG7 (HGNC:11237) |
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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 (PubMed:11549317, PubMed:28396416, PubMed:31097542, PubMed:9635427). 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 (By similarity). 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 (By similarity). 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 (By similarity). 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 (PubMed:28396416, PubMed:31097542). Also regulates mitochondrial calcium by catalyzing degradation of MCU (PubMed:31097542). Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function (PubMed:26387735). |
Cellular Location | Mitochondrion inner membrane; Multi-pass membrane protein |
Tissue Location | Ubiquitous. |
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Background
This gene encodes a nuclear-encoded mitochondrialmetalloprotease protein that is a member of the AAA (ATPasesassociated with a variety of cellular activities) protein family.Members of this protein family share an ATPase domain and haveroles in diverse cellular processes including membrane trafficking,intracellular motility, organelle biogenesis, protein folding, andproteolysis. Two transcript variants encoding distinct isoformshave been identified for this gene. Mutations associated with thisgene cause autosomal recessive spastic paraplegia 7. [provided byRefSeq].
References
Warnecke, T., et al. Mov. Disord. 25(4):413-420(2010)Augustin, S., et al. Mol. Cell 35(5):574-585(2009)Karlberg, T., et al. PLoS ONE 4 (10), E6975 (2009) :Brugman, F., et al. Neurology 71(19):1500-1505(2008)Tzoulis, C., et al. J. Neurol. 255(8):1142-1144(2008)
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