14631-1-AP

antibody from Proteintech Group

Targeting: AFG3L2 SCA28, SPAX5

Western blot (Supportive data in Antibodypedia)

ELISA (Data presented on provider website)

Immunocytochemistry (Supportive data in Antibodypedia)

Immunoprecipitation (Data presented on provider website)

Immunohistochemistry (Supportive data in Antibodypedia)

Antibody Data

Product number

14631-1-AP

Provider

Proteintech Group

Proper citation

Proteintech Cat#14631-1-AP, RRID:AB_2242420

Product name

AFG3L2 antibody

Antibody type

Polyclonal

Description

KD/KO validated AFG3L2 antibody (Cat. #14631-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat and has been validated for the following applications: IF, IHC, IP, WB,ELISA.

Reactivity

Human, Mouse, Rat

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

20ul, 150ul

References

Submitted references

Stress-induced OMA1-mediated cleavage of AIFM1 suppresses cell growth by controlling mitochondrial OXPHOS activity.

Nishigori M, Hirata S, Kosako H, Ichinohe T, Nolte H, Riemer J, Langer T, Koshiba T
The EMBO journal 2026 Mar 24;

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Physalin F Promotes AFG3L2-Mediated Degradation of VISA/MAVS to Suppress Innate Immune Response to RNA Virus.

Gao XN, Shu HB, Li M
Pathogens (Basel, Switzerland) 2026 Jan 9;15(1)

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CypD Dependent mPTP Opening Is Crucial for Oxidized Mitochondrial DNA Release in Ferroptosis.

Zhou H, Fu W, Liu S, Zhang Z, Luo H, Zhong Q
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2026 Apr;13(20):e02239

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FGF21 maintains redox homeostasis and promotes neuronal survival after traumatic brain injury by targeting SLC25A39-mediated mitochondrial GSH transport.

Wang L, Li W, Wu X, Ouyang Q, Sun B, Luo J, Guo H, Yang T, Ma Y
Journal of translational medicine 2025 Oct 2;23(1):1044

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Dual Role of CRABP2 in Colorectal Cancer: Oncogenesis via Nuclear RB1 and Cytoplasmic AFG3L2/SLC25A39 Axis, While Limiting Liver Metastasis through Cytoplasmic AFG3L2/PINK1/Parkin-Mediated Mitophagy.

Tian C, Yang S, Zhang C, Zhu R, Chen C, Wang X, Zhang D, Sun Q, Xu H, Nie H, Zhang Y, Ji D, Tang J, Jin K, Sun Y
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2025 Jun;12(23):e2500552

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TANGO2 binds crystallin alpha B and its loss causes desminopathy.

Stentenbach M, Hughes LA, Fagan SV, Payne B, Rudler DL, Siira SJ, McCubbin T, Chopin A, Perks KL, Ermer JA, Hendry J, Er TS, Balasubramaniam S, Eliades JA, Hool LC, Packer NH, Moh ESX, Padman BS, Rackham O, Filipovska A
Nature communications 2025 Jun 6;16(1):5261

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Mitochondrial dynamics and quality control regulate proteostasis in neuronal ischemia-reperfusion.

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Autophagy 2025 Jul;21(7):1492-1506

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The mitochondrial DNAJC co-chaperone TCAIM reduces α-ketoglutarate dehydrogenase protein levels to regulate metabolism.

Jiahui W, Xiang Y, Youhuan Z, Xiaomin M, Yuanzhu G, Dejian Z, Jie W, Yinkun F, Shi F, Juncheng S, Masha H, Marcia H, Peiyi W, Yingjie X, Wen Y
Molecular cell 2025 Feb 6;85(3):638-651.e9

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Proteolytic regulation of mitochondrial magnesium channel by m-AAA protease and prohibitin complex.

Joshi A, Stanfield RA, Spletter AT, Gohil VM
Genetics 2025 Feb 5;229(2)

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Leber's hereditary optic neuropathy-associated ND1 3733G>C mutation ameliorates the mitochondrial quality control and cellular homeostasis.

Yasheng M, Ji Y, He Y, Yi Q, Zhang H, Shan W, Wang K, Zhang J, Li Y, Meng F, Zhang M, Mo JQ, Wei S, Guan MX
The Journal of biological chemistry 2025 Aug;301(8):110464

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Pooled CRISPR Screening Identifies P-Bodies as Repressors of Cancer Epithelial-Mesenchymal Transition.

Fang L, Zhang L, Wang M, He Y, Yang J, Huang Z, Tan Y, Fang K, Li J, Sun Z, Li Y, Tang Y, Liang W, Cui H, Zhu Q, Wu Z, Li Y, Hu Y, Chen W
Cancer research 2024 Mar 4;84(5):659-674

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Dual regulation of SLC25A39 by AFG3L2 and iron controls mitochondrial glutathione homeostasis.

Shi X, DeCiucis M, Grabinska KA, Kanyo J, Liu A, Lam TT, Shen H
Molecular cell 2024 Feb 15;84(4):802-810.e6

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LonP1 Links Mitochondria-ER Interaction to Regulate Heart Function.

Li Y, Huang D, Jia L, Shangguan F, Gong S, Lan L, Song Z, Xu J, Yan C, Chen T, Tan Y, Liu Y, Huang X, Suzuki CK, Yang Z, Yang G, Lu B
Research (Washington, D.C.) 2023;6:0175

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FAM210A is essential for cold-induced mitochondrial remodeling in brown adipocytes.

Qiu J, Yue F, Zhu P, Chen J, Xu F, Zhang L, Kim KH, Snyder MM, Luo N, Xu HW, Huang F, Tao WA, Kuang S
Nature communications 2023 Oct 10;14(1):6344

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LPGAT1 controls MEGDEL syndrome by coupling phosphatidylglycerol remodeling with mitochondrial transport.

Sun H, Zhang J, Ye Q, Jiang T, Liu X, Zhang X, Zeng F, Li J, Zheng Y, Han X, Su C, Shi Y
Cell reports 2023 Nov 28;42(11):113214

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Ferroptosis inducers enhanced cuproptosis induced by copper ionophores in primary liver cancer.

Wang W, Lu K, Jiang X, Wei Q, Zhu L, Wang X, Jin H, Feng L
Journal of experimental & clinical cancer research : CR 2023 Jun 6;42(1):142

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Leber's hereditary optic neuropathy-associated ND6 14484T > C mutation caused pleiotropic effects on the complex I, RNA homeostasis, apoptosis and mitophagy.

Liang M, Ji Y, Zhang L, Wang X, Hu C, Zhang J, Zhu Y, Mo JQ, Guan MX
Human molecular genetics 2022 Sep 29;31(19):3299-3312

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Mechanisms and significance of tissue-specific MICU regulation of the mitochondrial calcium uniporter complex.

Tsai CW, Rodriguez MX, Van Keuren AM, Phillips CB, Shushunov HM, Lee JE, Garcia AM, Ambardekar AV, Cleveland JC Jr, Reisz JA, Proenza C, Chatfield KC, Tsai MF
Molecular cell 2022 Oct 6;82(19):3661-3676.e8

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Complementary anti-cancer pathways triggered by inhibition of sideroflexin 4 in ovarian cancer.

Tesfay L, Paul BT, Hegde P, Brewer M, Habbani S, Jellison E, Moore T, Wu H, Torti SV, Torti FM
Scientific reports 2022 Nov 19;12(1):19936

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Prohibitin 1 regulates mtDNA release and downstream inflammatory responses.

Liu H, Fan H, He P, Zhuang H, Liu X, Chen M, Zhong W, Zhang Y, Zhen C, Li Y, Jiang H, Meng T, Xu Y, Zhao G, Feng D
The EMBO journal 2022 Dec 15;41(24):e111173

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The mitochondrial UPR regulator ATF5 promotes intestinal barrier function via control of the satiety response.

Chamseddine D, Mahmud SA, Westfall AK, Castoe TA, Berg RE, Pellegrino MW
Cell reports 2022 Dec 13;41(11):111789

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A novel mutation located in the intermembrane space domain of AFG3L2 causes dominant optic atrophy through decreasing the stability of the encoded protein.

Yang L, Jin X, Li Y, Guo Q, Yang M, You Y, Yao S, Zhang X, Wang Z, Lei B
Cell death discovery 2022 Aug 15;8(1):361

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Translation of MT-ATP6 pathogenic variants reveals distinct regulatory consequences from the co-translational quality control of mitochondrial protein synthesis.

Ng KY, Richter U, Jackson CB, Seneca S, Battersby BJ
Human molecular genetics 2022 Apr 22;31(8):1230-1241

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A Mitochondrial DNA Variant Elevates the Risk of Gallstone Disease by Altering Mitochondrial Function.

Sun D, Niu Z, Zheng HX, Wu F, Jiang L, Han TQ, Wei Y, Wang J, Jin L
Cellular and molecular gastroenterology and hepatology 2021;11(4):1211-1226.e15

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Asymmetrical effects of deafness-associated mitochondrial DNA 7516delA mutation on the processing of RNAs in the H-strand and L-strand polycistronic transcripts.

Xiao Y, Wang M, He Q, Xu L, Zhang Q, Meng F, Jia Z, Zhang F, Wang H, Guan MX
Nucleic acids research 2020 Nov 4;48(19):11113-11129

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Overexpression of mitochondrial histidyl-tRNA synthetase restores mitochondrial dysfunction caused by a deafness-associated tRNA(His) mutation.

Gong S, Wang X, Meng F, Cui L, Yi Q, Zhao Q, Cang X, Cai Z, Mo JQ, Liang Y, Guan MX
The Journal of biological chemistry 2020 Jan 24;295(4):940-954

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Expanding the clinical and genetic heterogeneity of SPAX5.

Dosi C, Galatolo D, Rubegni A, Doccini S, Pasquariello R, Nesti C, Sicca F, Barghigiani M, Battini R, Tessa A, Santorelli FM
Annals of clinical and translational neurology 2020 Apr;7(4):595-601

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Sphingosine kinase 1 overexpression induces MFN2 fragmentation and alters mitochondrial matrix Ca(2+) handling in HeLa cells.

Pulli I, Löf C, Blom T, Asghar MY, Lassila T, Bäck N, Lin KL, Nyström JH, Kemppainen K, Toivola DM, Dufour E, Sanz A, Cooper HM, Parys JB, Törnquist K
Biochimica et biophysica acta. Molecular cell research 2019 Sep;1866(9):1475-1486

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Deletion of Gtpbp3 in zebrafish revealed the hypertrophic cardiomyopathy manifested by aberrant mitochondrial tRNA metabolism.

Chen D, Zhang Z, Chen C, Yao S, Yang Q, Li F, He X, Ai C, Wang M, Guan MX
Nucleic acids research 2019 Jun 4;47(10):5341-5355

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Mitochondrial stress response triggered by defects in protein synthesis quality control.

Richter U, Ng KY, Suomi F, Marttinen P, Turunen T, Jackson C, Suomalainen A, Vihinen H, Jokitalo E, Nyman TA, Isokallio MA, Stewart JB, Mancini C, Brusco A, Seneca S, Lombès A, Taylor RW, Battersby BJ
Life science alliance 2019 Feb;2(1)

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Fidelity of translation initiation is required for coordinated respiratory complex assembly.

Rudler DL, Hughes LA, Perks KL, Richman TR, Kuznetsova I, Ermer JA, Abudulai LN, Shearwood AJ, Viola HM, Hool LC, Siira SJ, Rackham O, Filipovska A
Science advances 2019 Dec;5(12):eaay2118

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Sideroflexin 4 affects Fe-S cluster biogenesis, iron metabolism, mitochondrial respiration and heme biosynthetic enzymes.

Paul BT, Tesfay L, Winkler CR, Torti FM, Torti SV
Scientific reports 2019 Dec 23;9(1):19634

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Mice harbouring a SCA28 patient mutation in AFG3L2 develop late-onset ataxia associated with enhanced mitochondrial proteotoxicity.

Mancini C, Hoxha E, Iommarini L, Brussino A, Richter U, Montarolo F, Cagnoli C, Parolisi R, Gondor Morosini DI, Nicolò V, Maltecca F, Muratori L, Ronchi G, Geuna S, Arnaboldi F, Donetti E, Giorgio E, Cavalieri S, Di Gregorio E, Pozzi E, Ferrero M, Riberi E, Casari G, Altruda F, Turco E, Gasparre G, Battersby BJ, Porcelli AM, Ferrero E, Brusco A, Tempia F
Neurobiology of disease 2019 Apr;124:14-28

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Sam50 Regulates PINK1-Parkin-Mediated Mitophagy by Controlling PINK1 Stability and Mitochondrial Morphology.

Jian F, Chen D, Chen L, Yan C, Lu B, Zhu Y, Chen S, Shi A, Chan DC, Song Z
Cell reports 2018 Jun 5;23(10):2989-3005

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Adult-onset obesity is triggered by impaired mitochondrial gene expression.

Perks KL, Ferreira N, Richman TR, Ermer JA, Kuznetsova I, Shearwood AJ, Lee RG, Viola HM, Johnstone VPA, Matthews V, Hool LC, Rackham O, Filipovska A
Science advances 2017 Aug;3(8):e1700677

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Proteolytic control of the mitochondrial calcium uniporter complex.

Tsai CW, Wu Y, Pao PC, Phillips CB, Williams C, Miller C, Ranaghan M, Tsai MF
Proceedings of the National Academy of Sciences of the United States of America 2017 Apr 25;114(17):4388-4393

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Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness.

Richter U, Lahtinen T, Marttinen P, Suomi F, Battersby BJ
The Journal of cell biology 2015 Oct 26;211(2):373-89

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Novel MTND1 mutations cause isolated exercise intolerance, complex I deficiency and increased assembly factor expression.

Gorman GS, Blakely EL, Hornig-Do HT, Tuppen HA, Greaves LC, He L, Baker A, Falkous G, Newman J, Trenell MI, Lecky B, Petty RK, Turnbull DM, McFarland R, Taylor RW
Clinical science (London, England : 1979) 2015 Jun;128(12):895-904

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Mutations in the SPG7 gene cause chronic progressive external ophthalmoplegia through disordered mitochondrial DNA maintenance.

Pfeffer G, Gorman GS, Griffin H, Kurzawa-Akanbi M, Blakely EL, Wilson I, Sitarz K, Moore D, Murphy JL, Alston CL, Pyle A, Coxhead J, Payne B, Gorrie GH, Longman C, Hadjivassiliou M, McConville J, Dick D, Imam I, Hilton D, Norwood F, Baker MR, Jaiser SR, Yu-Wai-Man P, Farrell M, McCarthy A, Lynch T, McFarland R, Schaefer AM, Turnbull DM, Horvath R, Taylor RW, Chinnery PF
Brain : a journal of neurology 2014 May;137(Pt 5):1323-36

Western blot

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

HeLa cells were subjected to SDS PAGE followed by western blot with 14631-1-AP(AFG3L2 antibody) at dilution of 1:800

Sample type

cell line

Immunocytochemistry

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

Immunofluorescent analysis of HepG2 cells, using AFG3L2 antibody 14631-1-AP at 1:25 dilution and Rhodamine-labeled goat anti-rabbit IgG (red).

Sample type

cell line

Immunohistochemistry

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

Immunohistochemical of paraffin-embedded human kidney using 14631-1-AP(AFG3L2 antibody) at dilution of 1:50 (under 10x lens)

Sample type

tissue

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

Immunohistochemical of paraffin-embedded human kidney using 14631-1-AP(AFG3L2 antibody) at dilution of 1:50 (under 40x lens)

Sample type

tissue