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The antibody against SPG7 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
The antibody against SPG7 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
| Cat.No | ADA-10909A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | SPG7 |
| Target Synonyms | CAR; PGN; CMAR; SPG5C; SPG7 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse brain, U-87 MG | Application | ELISA, WB, IF/ICC, IHC-P |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q9UQ90 | Immunogen Sequence |
Uniprot Id
Q9UQ90
Target Species
Human
Target Name
SPG7
Target Full Name
Mitochondrial inner membrane m-AAA protease component paraplegin
Target Function
ATP-dependent zinc metalloprotease. Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function.
Target Involvement
Spastic paraplegia 7, autosomal recessive (SPG7)
Target Subcellular Location
Mitochondrion inner membrane; Multi-pass membrane protein.
Target Protein Families
AAA ATPase family; Peptidase M41 family
Target Tissue Specificity
Ubiquitous.
Target Synonyms
SPG7; CAR; CMAR; PGN; Paraplegin; Cell matrix adhesion regulator; Spastic paraplegia 7 protein
Target 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.
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