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Paraplegin / SPG7
spastic paraplegia 7 (pure and complicated autosomal recessive)
Paraplegin / SPG7 is 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.
| Gene Name: | spastic paraplegia 7 (pure and complicated autosomal recessive) |
| Family/Subfamily: | Protease , Metallopeptidase M41 |
| Synonyms: | SPG7, Cell adhesion regulator, Cell matrix adhesion regulator, Paraplegin, PGN, SPG5C, Spastic paraplegia 7 protein, CAR, CMAR |
| Target Sequences: | NM_003119 NP_003110.1 Q9UQ90 |
Publications (1)
1
Impaired flickering of the permeability transition pore causes SPG7 spastic paraplegia. Irene Sambri, Filomena Massa, Francesca Gullo, Simone Meneghini, Laura Cassina, Michela Carraro, Giorgia Dina, Angelo Quattrini, Lorenzo Patanella, Annamaria Carissimo, Antonella Iuliano, Filippo Santorelli, Franca Codazzi, Fabio Grohovaz, Paolo Bernardi, Andrea Becchetti, Giorgio Casari. EBioMedicine. 2020 November;61:103050. [Full Text Article]
[PubMed:33045469]
[PMC:PMC7553352]
Related Antibodies: LS-C175082.
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(43)
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(20)
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Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI3H10) Antibody
Human
Flo, IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (Carrier-free) (OTI3H10) Antibody
Human
Flo, IF, WB
Unconjugated, Carrier-free
100 µg/$511
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) Antibody
Human
IHC, IHC-P, WB
Unconjugated
400 µl/$393
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (OTI1D3) Antibody
Human
Flo, IHC, IHC-P, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (Carrier-free) (OTI1D3) Antibody
Human
Flo, IHC, WB
Unconjugated, Carrier-free
100 µg/$511
Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI1C12) Antibody
Mouse, Human
Flo, IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (Carrier-free) (OTI1C12) Antibody
Mouse, Human
Flo, IF, WB
Unconjugated, Carrier-free
100 µg/$511
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (OTI1C1) Antibody
Mouse, Dog, Rat, Human, Monkey
Flo, IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (Carrier-free) (OTI1C1) Antibody
Mouse, Dog, Rat, Human, Monkey
Flo, IF, WB
Unconjugated, Carrier-free
100 µg/$511
Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI7D10) Antibody
Human
Flo, IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (Carrier-free) (OTI7D10) Antibody
Human
Flo, IF, WB
Unconjugated, Carrier-free
100 µg/$511
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (OTI1G4) Antibody
Human
IHC, IHC-P, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (Carrier-free) (OTI1G4) Antibody
Human
IHC, WB
Unconjugated, Carrier-free
100 µg/$511
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) Antibody
Human
ELISA, IHC, WB
Unconjugated
200 µl/$734
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) (Biotin) Antibody
Human
ELISA, IHC, WB
Biotin Conjugated
200 µl/$952
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) (FITC) Antibody
Human
ELISA, IHC, WB
FITC Conjugated
200 µl/$952
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) (Azide-free) (HRP) Antibody
Human
ELISA, IHC, WB
HRP Conjugated, Azide-free
200 µl/$952
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) (APC) Antibody
Human
ELISA, IHC, WB
APC Conjugated
200 µl/$952
Paraplegin / SPG7 Rabbit anti-Human Polyclonal (aa115-141) (PE) Antibody
Human
ELISA, IHC, WB
PE Conjugated
200 µl/$952
Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI1D10) Antibody
Human
IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI3E8) Antibody
Human
IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI5H7) Antibody
Human
IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (OTI1H4) Antibody
Human
IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (OTI1E5) Antibody
Human
IF, WB
Unconjugated
100 µl/$434
Paraplegin / SPG7 Mouse anti-Human Monoclonal (aa300-573) (OTI2B9) Antibody
Human
IF, WB
Unconjugated
100 µl/$434
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