SPG7 Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| IHC-P, WB, E |
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Primary Accession | Q9UQ90 |
Other Accession | NP_955399.1, NP_003110.1 |
Reactivity | Human |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | Rabbit IgG |
Calculated MW | 88235 Da |
Antigen Region | 115-141 aa |
Gene ID | 6687 |
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Other Names | Paraplegin, 3424-, Spastic paraplegia 7 protein, SPG7, CAR, CMAR, PGN |
Target/Specificity | This SPG7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 115-141 amino acids from the Central region of human SPG7. |
Dilution | WB~~1:1000 IHC-P~~1:10~50 |
Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
Precautions | SPG7 Antibody (Center) is for research use only and 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 mitochondrial metalloprotease protein that is a member of the AAA (ATPases associated with a variety of cellular activities) protein family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Two transcript variants encoding distinct isoforms have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 7. [provided by RefSeq].
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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