Rabbit Anti-SPG7 Polyclonal Antibody
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产品编号
PAV3786
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别名
SPG7 matrix AAA peptidase subunit, paraplegin, CAR, CMAR, PGN, SPG5C
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目录价格
InquiryRMB
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规格
- 100ul
产品介绍
| Alias | SPG7 matrix AAA peptidase subunit, paraplegin, CAR, CMAR, PGN, SPG5C |
| Catalog Number | PAV3786 |
| Product Description | Rabbit polyclonal antibody raised against human SPG7 (Mitochondrial inner membrane m-AAA protease component paraplegin). Affinity purified from rabbit antiserum by antigen-specific affinity chromatography. Validated applications: WB, ELISA. Supplied as liquid in PBS containing 50% glycerol and 0.02% sodium azide at 1 mg/ml. |
| Molecular Name | SPG7 |
| Species | Human |
| Host | Rabbit |
| Size | 100ul |
| Clonity | Polyclonal |
| Protein Family | 蛋白酶 |
| Concentration | 1 mg/ml |
| Purification | affinity-chromatography |
| Formulation | supply in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Background | This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA 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. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Mar 2014] |
| Immunogen | This antibody was raised against from part region of human SPG7 protein (UniProt: Q9UQ90) |
| Application | WB, ELISA |
| Recommended Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| Gene ID | 6687 |
| Tag | Unconjugated |
| Cross-reactivity | Human |
| Predicted Molecular Weight | ≈88.2 kDa |
| Storage Condition | Store at 4℃ for for short term storage. It is recommended to aliquote to ~10ul per vial and store at -20℃ for long term storage. Avoid repeated freezing and thawing cycles. |
| Shipping Condition | This product is shipped on blue ice. |
| Molecular Weight | 88.2 kDa |
| Cross-reactivity | Human |
| Uniprot ID | Q9UQ90 |
| Note | Gene ID: 6687; UniProt: Q9UQ90; Chromosome: 16q24.3; Predicted MW: 88.2 kDa |
| 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 (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. |
| Product Declaration | 本产品仅用于研究用途(RUO)。不能用于诊断或直接用于人体实验。 |
| Product Full Name | Rabbit Anti-Human SPG7 Polyclonal Antibody |
| Target | SPG7 |
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