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AB68023

Anti-AFG3L2 antibody

3

(1 Review)

|

(6 Publications)

Mouse Polyclonal AFG3L2 antibody. Suitable for WB, ICC/IF and reacts with Human samples. Cited in 6 publications. Immunogen corresponding to Recombinant Full Length Protein corresponding to Human AFG3-like protein 2.

View Alternative Names

Mitochondrial inner membrane m-AAA protease component AFG3L2, AFG3-like protein 2, Paraplegin-like protein, AFG3L2

2 Images
Immunocytochemistry/ Immunofluorescence - Anti-AFG3L2 antibody (AB68023)
  • ICC/IF

Unknown

Immunocytochemistry/ Immunofluorescence - Anti-AFG3L2 antibody (AB68023)

ICC/IF image of ab68023 stained HepG2 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab68023, 5µg/ml) overnight at +4°C. The secondary antibody (green) was ab96879, DyLight® 488 goat anti-mouse IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

Western blot - Anti-AFG3L2 antibody (AB68023)
  • WB

Unknown

Western blot - Anti-AFG3L2 antibody (AB68023)

All lanes:

Western blot - Anti-AFG3L2 antibody (ab68023) at 1/500 dilution

Lane 1:

AFG3L2 transfected 293T cell lysate at 25 µg

Lane 2:

Non transfected 293T cell lysate at 25 µg

Secondary

All lanes:

Goat Anti-Mouse IgG (H&L)-HRP Conjugate secondaryantibody at 1/2500 dilution

Predicted band size: 89 kDa

Observed band size: 80 kDa

true

Key facts

Host species

Mouse

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

WB, ICC/IF

applications

Immunogen

Recombinant Full Length Protein corresponding to Human AFG3-like protein 2.

Q9Y4W6

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/500 - 1/1000", "WB-species-notes": "<p></p>", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "5 µg/mL", "ICCIF-species-notes": "<p></p>" } } }
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Properties and storage information

Form
Liquid
Purification technique
Affinity purification Protein A
Storage buffer
pH: 7.4 Constituents: PBS
Shipped at conditions
Blue Ice
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
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Supplementary information

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

AFG3L2 also known as AFGL2 is a mitochondrial protein with a mass of approximately 90 kDa. It belongs to the AAA protease family and functions as a metalloprotease. You can find AFG3L2 expressed mainly in the brain heart and skeletal muscles. It resides in the inner mitochondrial membrane where it plays a mechanical role in protein quality control and processing eliminating misfolded or damaged proteins within the mitochondria.
Biological function summary

AFG3L2 is important for maintaining mitochondrial integrity and functional protein homeostasis. AFG3L2 forms a part of the m-AAA protease complex along with its homolog SPG7. This complex is integral for the regulation of mitochondrial ribosome assembly and is involved in regulating the mitochondrial respiratory chain complexes. AFG3L2’s role in processing several protein precursors helps ensure efficient mitochondrial function highlighting its importance in maintaining cellular energy balance.

Pathways

AFG3L2’s involvement spans across critical mitochondrial processes including the OXPHOS (oxidative phosphorylation) pathway and mitochondrial biogenesis. The activity of AFG3L2 affects key proteins like OPA1 which highlights its role in mitochondrial dynamics and energy metabolism. Moreover this protein’s interactions also influence the activity and stability of diverse respiratory chain complexes establishing it as an important player in cellular energy production.

AFG3L2 mutations have been linked to spinocerebellar ataxia type 28 (SCA28) and optic atrophy highlighting its relevance to neurodegenerative conditions. These conditions result from disruptions in mitochondrial function emphasizing the importance of AFG3L2 in neuronal health. It also connects with proteins such as paraplegin with which it exerts synergistic effects and abnormalities in this interaction often worsen the expression and impact of both SCA28 and related pathologies.
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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
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Target data

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 : 19748354, PubMed : 28396416, PubMed : 29932645, PubMed : 30683687, PubMed : 31327635, PubMed : 37917749, PubMed : 38157846). AFG3L2 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 (PubMed : 19748354, PubMed : 31327635). The m-AAA protease carries out quality control in the inner membrane of the mitochondria by mediating degradation of mistranslated or misfolded polypeptides (PubMed : 26504172, PubMed : 30683687, PubMed : 34718584). The m-AAA protease complex also promotes the processing and maturation of mitochondrial proteins, such as MRPL32/bL32m, PINK1 and SP7 (PubMed : 22354088, PubMed : 29932645, PubMed : 30252181). 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 (PubMed : 29932645). Required for SPG7 maturation into its active mature form after SPG7 cleavage by mitochondrial-processing peptidase (MPP) (PubMed : 30252181). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed : 22354088). 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 : 27642048, PubMed : 28396416). Promotes the proteolytic degradation of GHITM upon hyperpolarization of mitochondria : progressive GHITM degradation leads to respiratory complex I degradation and broad reshaping of the mitochondrial proteome by AFG3L2 (PubMed : 35912435). Also acts as a regulator of mitochondrial glutathione homeostasis by mediating cleavage and degradation of SLC25A39 (PubMed : 37917749, PubMed : 38157846). SLC25A39 cleavage is prevented when SLC25A39 binds iron-sulfur (PubMed : 37917749, PubMed : 38157846). Involved in the regulation of OMA1-dependent processing of OPA1 (PubMed : 17615298, PubMed : 29545505, PubMed : 30252181, PubMed : 30683687, PubMed : 32600459). May act by mediating processing of OMA1 precursor, participating in OMA1 maturation (PubMed : 29545505).
See full target information AFG3-like protein 2
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Publications (6)

Recent publications for all applications. Explore the full list and refine your search

Investigative ophthalmology & visual science 62:38 PubMed34311469

2021

Assocation Between Leber's Hereditary Optic Neuropathy and MT-ND1 3460G>A Mutation-Induced Alterations in Mitochondrial Function, Apoptosis, and Mitophagy.

Applications

Unspecified application

Species

Unspecified reactive species

Juanjuan Zhang,Yanchun Ji,Jie Chen,Man Xu,Guoping Wang,Xiaorui Ci,Bing Lin,Jun Q Mo,Xiangtian Zhou,Min-Xin Guan

International journal of molecular sciences 19: PubMed30544562

2018

Loss of Mitochondrial AAA Proteases AFG3L2 and YME1L Impairs Mitochondrial Structure and Respiratory Chain Biogenesis.

Applications

Unspecified application

Species

Unspecified reactive species

Jana Cesnekova,Marie Rodinova,Hana Hansikova,Jiri Zeman,Lukas Stiburek

Scientific reports 8:1163 PubMed29348686

2018

Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis.

Applications

Unspecified application

Species

Unspecified reactive species

Rachid Boutoual,Salvador Meseguer,Magda Villarroya,Elena Martín-Hernández,Mohammed Errami,Miguel A Martín,Marta Casado,M-Eugenia Armengod

Scientific reports 7:6209 PubMed28740091

2017

microRNA-mediated differential expression of TRMU, GTPBP3 and MTO1 in cell models of mitochondrial-DNA diseases.

Applications

Unspecified application

Species

Unspecified reactive species

Salvador Meseguer,Olga Boix,Carmen Navarro-González,Magda Villarroya,Rachid Boutoual,Sonia Emperador,Elena García-Arumí,Julio Montoya,M-Eugenia Armengod

Proceedings of the National Academy of Sciences of 111:14782-7 PubMed25275009

2014

Activation of mitochondrial protease OMA1 by Bax and Bak promotes cytochrome c release during apoptosis.

Applications

Unspecified application

Species

Unspecified reactive species

Xian Jiang,Hui Jiang,Zhirong Shen,Xiaodong Wang

Journal of cell science 127:2313-25 PubMed24634514

2014

Impaired OMA1-dependent cleavage of OPA1 and reduced DRP1 fission activity combine to prevent mitophagy in cells that are dependent on oxidative phosphorylation.

Applications

Unspecified application

Species

Unspecified reactive species

Thomas D B MacVicar,Jon D Lane
View all publications
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