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Rabbit Recombinant Monoclonal SPG7/PGN antibody. Suitable for WB, IP and reacts with Human, Mouse, Rat samples.

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Images

Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (AB305255), expandable thumbnail
  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (AB305255), expandable thumbnail
  • Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (AB305255), expandable thumbnail
  • Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (AB305255), expandable thumbnail
  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (AB305255), expandable thumbnail

Key facts

Isotype
IgG
Host species
Rabbit
Storage buffer

pH: 7.2 - 7.4
Preservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA

Form
Liquid
Clonality
Monoclonal

Immunogen

  • The exact immunogen used to generate this antibody is proprietary information.

Reactivity data

Select an application
Product promiseTestedExpectedPredictedNot recommended
WBIHC-PICC/IFFlow Cyt (Intra)IP
Human
Tested
Not recommended
Not recommended
Not recommended
Tested
Mouse
Tested
Not recommended
Not recommended
Not recommended
Tested
Rat
Tested
Not recommended
Not recommended
Not recommended
Expected

Tested
Tested

Species
Human
Dilution info
1/1000
Notes

-

Species
Mouse
Dilution info
1/1000
Notes

-

Species
Rat
Dilution info
1/1000
Notes

-

Not recommended
Not recommended

Species
Human
Dilution info
-
Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Species
Mouse
Dilution info
-
Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Species
Rat
Dilution info
-
Notes

Perform heat-mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

Not recommended
Not recommended

Species
Human, Mouse, Rat
Dilution info
-
Notes

-

Not recommended
Not recommended

Species
Human, Mouse, Rat
Dilution info
-
Notes

-

Tested
Tested

Species
Human
Dilution info
1/30
Notes

-

Species
Mouse
Dilution info
1/30
Notes

-

Expected
Expected

Species
Rat
Dilution info
Use at an assay dependent concentration.
Notes

-

Target data

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).

Alternative names

CAR, CMAR, PGN, SPG7, Mitochondrial inner membrane m-AAA protease component paraplegin, Cell matrix adhesion regulator, Paraplegin, Spastic paraplegia 7 protein

Recommended products

Rabbit Recombinant Monoclonal SPG7/PGN antibody. Suitable for WB, IP and reacts with Human, Mouse, Rat samples.

Key facts

Isotype
IgG
Form
Liquid
Clonality
Monoclonal
Immunogen
  • The exact immunogen used to generate this antibody is proprietary information.
Clone number
EPR26225-9
Purification technique
Affinity purification Protein A
Concentration
Loading...

Storage

Shipped at conditions
Blue Ice
Appropriate short-term storage duration
1-2 weeks
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle

Notes

Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.

This product is a recombinant monoclonal antibody, which offers several advantages including:

  • - High batch-to-batch consistency and reproducibility
  • - Improved sensitivity and specificity
  • - Long-term security of supply
  • - Animal-free batch production

For more information, read more on recombinant antibodies.

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.
Activity summary

SPG7 also known as paraplegin is a mitochondrial metalloprotease. It has a molecular mass of approximately 88 kDa. SPG7 is encoded by the SPG7 gene located in humans on chromosome 16. It expresses widely in tissues with a strong presence in the nervous system and muscles. Alternate names for this protein are PGN and spastic paraplegia 7 due to its association with specific neurodegenerative conditions.

Biological function summary

SPG7 functions as a part of the mitochondrial inner membrane m-AAA protease complex. This complex performs essential roles in the maintenance of mitochondrial homeostasis by degrading damaged or misfolded proteins within the mitochondria. The presence of SPG7 is important for proper mitochondrial function. The protein has proteolytic activity which supports mitochondrial respiratory chain complexes' assembly and stability.

Pathways

SPG7 directly involves itself in mitochondrial protein quality control and the broader regulation of mitochondrial function. It interacts closely with ATP-dependent proteases like AFG3L2 playing a significant part in mitochondrial biogenesis pathways. These partnerships are key for maintaining cellular energy balance and ensuring efficient electron transport chain operation.

Associated diseases and disorders

SPG7 mutations connect predominantly to hereditary spastic paraplegia (HSP) and have also been linked to ataxia. These mutations interrupt normal mitochondrial function leading to the degeneration of motor pathways. In HSP SPG7 interacts with proteins such as paraplegin-associated proteins SPG5 and SPG11 contributing to the disorder's pathogenesis. Understanding SPG7’s role in these diseases aids in exploring therapeutic strategies for mitochondrial-related disorders.

Product promise

We are dedicated to supporting your work with high quality reagents and we are here for you every step of the way should you need us.

In the unlikely event of one of our products not working as expected, you are covered by our product promise.

Full details and terms and conditions can be found here:
Terms & Conditions.

7 product images

  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST.

    In Western blot, anti-GAPDH antibody (Anti-GAPDH antibody [EPR16891] - Loading Control ab181602) loading control staining at 1/200000 dilution. Anti-SPG7 (ab305255) staining at 1/200000 dilution.

    Exposure time: 59 seconds.

    All lanes: Western blot - Anti-CHCHD10 antibody [EPR26966-33] (Anti-CHCHD10 antibody [EPR26966-33] ab308510) at 1/1000 dilution

    Lane 1: HeLa (human cervical adenocarcinoma epithelial cell) non-mitochondrial fraction at 20 µg

    Lane 2: HeLa mitochondrial fraction at 20 µg

    Secondary

    All lanes: Goat Anti-Rabbit IgG (HRP) with minimal cross-reactivity with human IgG at 1/2000 dilution

    Predicted band size: 14 kDa

    Observed band size: 18 kDa

    Exposure time: 59s

  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST.

    This blot was developed using a high sensitivity ECL substrate.

    In Western blot, anti-SPG7 antibody (ab305255) staining at 1/1000 dilution. Anti-GAPDH antibody (Anti-GAPDH antibody [EPR16891] - Loading Control ab181602) loading control staining at 1/200000 dilution.

    Exposure time: 92 seconds.

    All lanes: Western blot - Anti-CHCHD10 antibody [EPR26493-17] (Anti-CHCHD10 antibody [EPR26493-17] ab308508) at 1/1000 dilution

    Lane 1: Mouse liver non-mitochondrial fraction at 20 µg

    Lane 2: Mouse liver mitochondria fraction at 20 µg

    Secondary

    All lanes: Goat Anti-Rabbit IgG (HRP) with minimal cross-reactivity with human IgG at 1/2000 dilution

    Predicted band size: 14 kDa

    Observed band size: 15 kDa

    Exposure time: 92s

  • Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    SPG7/PGN was immunoprecipitated from 0.35 mg NIH/3T3 (mouse embryonic fibroblast) whole cell lysate 10 μg with ab305255 at 1/30 dilution (2μg in 0.35mg lysates). Western blot was performed on the immunoprecipitate using ab305255. VeriBlot for IP secondary antibody(HRP)(VeriBlot for IP Detection Reagent (HRP) ab131366) was used at 1/5000 dilution.

    Lane 1: NIH/3T3 (mouse embryonic fibroblast) whole cell lysate 10 μg

    Lane 2: abab305255 IP in NIH/3T3 whole cell lysate

    Lane 3: Rabbit monoclonal IgG (Rabbit IgG, monoclonal [EPR25A] - Isotype Control ab172730) instead of ab305255 in NIH/3T3 whole cell lysate

    Blocking and dilution buffer and concentration: 5% NFDM/TBST.

    Exposure time: 32 seconds

    All lanes: Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255) at 1/30 dilution

    All lanes: NIH/3T3 (mouse embryonic fibroblast)

    Secondary

    All lanes: Immunoprecipitation - VeriBlot for IP Detection Reagent (HRP) (VeriBlot for IP Detection Reagent (HRP) ab131366) at 1/5000 dilution

    Observed band size: 88 kDa

    Exposure time: 32s

  • Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    SPG7/PGN was immunoprecipitated from 0.35 mg HEK-293 (human embryonic kidney epithelial cell) whole cell lysate 10 μg with ab305255 at 1/30 dilution (2μg in 0.35mg lysates). VeriBlot for IP secondary antibody(HRP)(VeriBlot for IP Detection Reagent (HRP) ab131366) was used at 1/5000 dilution.

    Lane 1: HEK-293 (human embryonic kidney epithelial cell) whole cell lysate 10 μg

    Lane 2: ab305255 IP in HEK-293 whole cell lysate

    Lane 3: Rabbit monoclonal IgG (Rabbit IgG, monoclonal [EPR25A] - Isotype Control ab172730) instead of ab305255 in HEK-293 whole cell lysate

    Blocking and dilution buffer and concentration: 5% NFDM/TBST.

    Exposure time: 3 minutes

    All lanes: Immunoprecipitation - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255) at 1/30 dilution

    All lanes: HEK-293 (human embryonic kidney epithelial cell)

    Secondary

    All lanes: Immunoprecipitation - VeriBlot for IP Detection Reagent (HRP) (VeriBlot for IP Detection Reagent (HRP) ab131366) at 1/5000 dilution

    Observed band size: 88 kDa

    Exposure time: 3min

  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST
    Exposure time: 3 minutes

    All lanes: Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255) at 1/1000 dilution

    Lane 1: HepG2 (human hepatocellar carcinoma epithelial cell), whole cell lysate at 20 µg

    Lane 2: Neuro-2a (mouse neuroblastoma neuroblast), whole cell lysate at 20 µg

    Lane 3: MEF (mouse embryonic fibroblast (immortalized)), whole cell lysate at 20 µg

    Lane 4: NIH/3T3 (mouse embryonic fibroblast), whole cell lysate at 20 µg

    Lane 5: PC-12 (rat adrenal gland pheochromocytoma), whole cell lysate at 20 µg

    Lane 6: Mouse cerebellum tissue lysate at 20 µg

    Lane 7: Mouse heart tissue lysate at 20 µg

    Lane 8: Rat cerebellum tissue lysate at 20 µg

    Secondary

    All lanes: Western blot - Goat Anti-Rabbit IgG H&L (HRP) (Goat Anti-Rabbit IgG H&L (HRP) ab97051) at 1/50000 dilution

    Observed band size: 88 kDa

    Exposure time: 3min

  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST
    Exposure time: 26 seconds

    All lanes: Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255) at 1/1000 dilution

    Lane 1: HeLa (human cervix adenocarcinoma epithelial cell) non-mitochondrial fraction at 20 µg

    Lane 2: HeLa mitochondria fraction at 20 µg

    Secondary

    All lanes: Western blot - Goat Anti-Rabbit IgG H&L (HRP) (Goat Anti-Rabbit IgG H&L (HRP) ab97051) at 1/50000 dilution

    Observed band size: 88 kDa

    Exposure time: 26s

  • Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255), expandable thumbnail

    Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255)

    Blocking and diluting buffer and concentration: 5% NFDM/TBST
    Exposure time: 3 minutes

    All lanes: Western blot - Anti-SPG7/PGN antibody [EPR26225-9] (ab305255) at 1/1000 dilution

    Lane 1: HEK-293T (human embryonic kidney epithelial cell) transfected with scrambled siRNA control, whole cell lysate at 20 µg

    Lane 2: HEK-293T transfected with siRNA specifically targeting SPG7/PGN, whole cell lysate at 20 µg

    Secondary

    All lanes: Western blot - Goat Anti-Rabbit IgG H&L (HRP) (Goat Anti-Rabbit IgG H&L (HRP) ab97051) at 1/50000 dilution

    Observed band size: 88 kDa

    Exposure time: 3min

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Product protocols

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