19424-1-AP

antibody from Proteintech Group

Targeting: CHCHD2 C7orf17, MIX17B, MNRR1

Provider product page for 19424-1-AP

Western blot

ELISA

Immunocytochemistry

Immunoprecipitation

Immunohistochemistry

Antibody data

Antibody Data
Antigen structure
References [48]
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Validations
Western blot [1]
Immunocytochemistry [1]

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Product number: 19424-1-AP - Provider product page
Provider: Proteintech Group
Proper citation: Proteintech Cat#19424-1-AP, RRID:AB_10638907
Product name: CHCHD2 antibody
Antibody type: Polyclonal
Description: KD/KO validated CHCHD2 antibody (Cat. #19424-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse and has been validated for the following applications: IF, IHC, IP, WB,ELISA.
Reactivity: Human, Mouse
Host: Rabbit
Conjugate: Unconjugated
Isotype: IgG
Vial size: 20ul, 150ul

CHCHD2 protein structure - 19424-1-AP shown in red.

Submitted references CHCHD2 mutant mice link mitochondrial deficits to PD pathophysiology.

Liao SC, Kano K, Phanse S, Nguyen M, Margolis E, Fu Y, X Meng J, Moutaoufik MT, Chatterton Z, Saccon T, Broderick K, Aoki H, Simms J, Suteja FX, Sei Y, Huang EJ, McAvoy K, Manfredi G, Halliday G, Babu M, Nakamura K
Science advances 2025 Nov 14;11(46):eadu0726

CHCHD2 rescues the mitochondrial dysfunction in iPSC-derived neurons from patient with Mohr-Tranebjaerg syndrome.

Huang Y, Chen Z, Deng W, Jiang Y, Pan Y, Yuan Z, Hu H, Wu Y, Hu Y
Cell death & disease 2025 Mar 12;16(1):173

The Efficacy of Hellebrigenin Against Nasopharyngeal Carcinoma Cells: The Molecular and Bioinformatic Analysis.

Ho HY, Chen MK, Tsai YJ, Lin CC, Lo YS, Chuang YC, Hsieh MJ
Journal of cellular and molecular medicine 2025 Jun;29(11):e70624

Therapeutic Potential of CHCHD2 in Ischemia-Reperfusion Injury: Mechanistic Insights into Nrf2-Dependent Antioxidant Defense in HK2 Cells.

Hao Y, Zhou X
International journal of molecular sciences 2025 Jun 25;26(13)

LncRNA MALAT1 Facilitates HIV-1 Replication by Upregulation of CHCHD2 and Downregulation of IFN-I Expression.

Wang MR, Bai CS, Dai JW, Yang L, Quan FY, Ma JC, Chen XY, Zhu SW, Xu YQ, Xiang ZF, Jiang YL, Cheng Q, Zhang WH, Chen KH, Wang JH, Feng Y, Chen XP, Xiong Y, Chen SL, Hou W, Xiong HR
Molecular & cellular proteomics : MCP 2025 Jul;24(7):100997

Pseudohypoxia-Stabilized HIF2α Transcriptionally Inhibits MNRR1, a Druggable Target in MELAS.

Purandare N, Pasupathi V, Xi Y, Rajan V, Vegh C, Firestine S, Kozicz T, Fribley AM, Grossman LI, Aras S
Cells 2025 Jul 15;14(14)

Neuroprotective role of CHCHD2 in Parkinson's disease: Insights into the GPX4-related ferroptosis pathway.

Wang F, Liu X, Chen M, Xu X, Yang Y, Xu Q, Zhu H, Xu A, Pouladi MA, Xu X
Free radical biology & medicine 2025 Jan;226:348-363

Clioquinol induces mitochondrial toxicity in SH-SY5Y neuroblastoma cells by affecting the respiratory chain complex IV and OPA1 dynamin-like GTPase.

Katsuyama M, Arakawa N, Yaoi T, Kimura E, Matsumoto M, Iwata K, Umemura A, Yabe-Nishimura C
FEBS letters 2025 Apr;599(8):1135-1145

MIA40 suppresses cell death induced by apoptosis-inducing factor 1.

Mussulini BHM, Maruszczak KK, Draczkowski P, Borrero-Landazabal MA, Ayyamperumal S, Wnorowski A, Wasilewski M, Chacinska A
EMBO reports 2025 Apr;26(7):1835-1862

Loss of CHCHD2 Stability Coordinates with C1QBP/CHCHD2/CHCHD10 Complex Impairment to Mediate PD-Linked Mitochondrial Dysfunction.

Ren YL, Jiang Z, Wang JY, He Q, Li SX, Gu XJ, Qi YR, Zhang M, Yang WJ, Cao B, Li JY, Wang Y, Chen YP
Molecular neurobiology 2024 Oct;61(10):7968-7988

High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice.

Southwell N, Manzo O, Bacman S, Zhao D, Sayles NM, Dash J, Fujita K, D'Aurelio M, Di Lorenzo A, Manfredi G, Kawamata H
EMBO molecular medicine 2024 Jun;16(6):1352-1378

CHCHD2 Thr61Ile mutation impairs F1F0-ATPase assembly in in vitro and in vivo models of Parkinson's disease.

Chen X, Lin Y, Zhang Z, Tang Y, Ye P, Dai W, Zhang W, Liu H, Peng G, Huang S, Qiu J, Guo W, Zhu X, Wu Z, Kuang Y, Xu P, Zhou M
Neural regeneration research 2024 Jan;19(1):196-204

Epigenetic repression of CHCHD2 enhances survival from single cell dissociation through attenuated Rho A kinase activity.

Kim J, Kwon EJ, Kim YJ, Kim D, Shin YZ, Gil D, Kim JH, Shin HD, Kim LH, Lee MO, Go YH, Cha HJ
Cellular and molecular life sciences : CMLS 2024 Jan 12;81(1):38

Genetic and pharmacologic p32-inhibition rescue CHCHD2-linked Parkinson's disease phenotypes in vivo and in cell models.

Tio M, Wen R, Choo CN, Tan JB, Chua A, Xiao B, Sundaram JR, Chan CHS, Tan EK
Journal of biomedical science 2024 Feb 23;31(1):24

CHCHD2 up-regulation in Huntington disease mediates a compensatory protective response against oxidative stress.

Liu X, Wang F, Fan X, Chen M, Xu X, Xu Q, Zhu H, Xu A, Pouladi MA, Xu X
Cell death & disease 2024 Feb 10;15(2):126

CHCHD2 mutant mice display mitochondrial protein accumulation and disrupted energy metabolism.

Liao SC, Kano K, Phanse S, Nguyen M, Margolis E, Fu Y, Meng J, Moutaoufik MT, Chatterton Z, Aoki H, Simms J, Hsieh I, Suteja F, Sei Y, Huang EJ, McAvoy K, Manfredi G, Halliday G, Babu M, Nakamura K
bioRxiv : the preprint server for biology 2024 Aug 31;

Mutant huntingtin impairs neurodevelopment in human brain organoids through CHCHD2-mediated neurometabolic failure.

Lisowski P, Lickfett S, Rybak-Wolf A, Menacho C, Le S, Pentimalli TM, Notopoulou S, Dykstra W, Oehler D, López-Calcerrada S, Mlody B, Otto M, Wu H, Richter Y, Roth P, Anand R, Kulka LAM, Meierhofer D, Glazar P, Legnini I, Telugu NS, Hahn T, Neuendorf N, Miller DC, Böddrich A, Polzin A, Mayatepek E, Diecke S, Olzscha H, Kirstein J, Ugalde C, Petrakis S, Cambridge S, Rajewsky N, Kühn R, Wanker EE, Priller J, Metzger JJ, Prigione A
Nature communications 2024 Aug 22;15(1):7027

Involvement of casein kinase 1 epsilon/delta (Csnk1e/d) in the pathogenesis of familial Parkinson's disease caused by CHCHD2.

Torii S, Arakawa S, Sato S, Ishikawa KI, Taniguchi D, Sakurai HT, Honda S, Hiraoka Y, Ono M, Akamatsu W, Hattori N, Shimizu S
EMBO molecular medicine 2023 Sep 11;15(9):e17451

The CHCHD2/Sirt1 corepressors involve in G9a-mediated regulation of RNase H1 expression to control R-loop.

Li L, Wu Y, Dai K, Wang Q, Ye S, Shi Q, Chen Z, Huang YC, Zhao W, Li L
Cell insight 2023 Aug;2(4):100112

MICU1 regulates mitochondrial cristae structure and function independently of the mitochondrial Ca(2+) uniporter channel.

Tomar D, Thomas M, Garbincius JF, Kolmetzky DW, Salik O, Jadiya P, Joseph SK, Carpenter AC, Hajnóczky G, Elrod JW
Science signaling 2023 Apr 25;16(782):eabi8948

Lipopolysaccharide induces placental mitochondrial dysfunction in murine and human systems by reducing MNRR1 levels via a TLR4-independent pathway.

Purandare N, Kunji Y, Xi Y, Romero R, Gomez-Lopez N, Fribley A, Grossman LI, Aras S
iScience 2022 Nov 18;25(11):105342

Mouse midbrain dopaminergic neurons survive loss of the PD-associated mitochondrial protein CHCHD2.

Nguyen MK, McAvoy K, Liao SC, Doric Z, Lo I, Li H, Manfredi G, Nakamura K
Human molecular genetics 2022 May 4;31(9):1500-1518

Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy.

Sayles NM, Southwell N, McAvoy K, Kim K, Pesini A, Anderson CJ, Quinzii C, Cloonan S, Kawamata H, Manfredi G
Cell reports 2022 Mar 8;38(10):110475

CHCHD2 and CHCHD10 regulate mitochondrial dynamics and integrated stress response.

Ruan Y, Hu J, Che Y, Liu Y, Luo Z, Cheng J, Han Q, He H, Zhou Q
Cell death & disease 2022 Feb 16;13(2):156

Human Induced Pluripotent Stem Cell-Derived TDP-43 Mutant Neurons Exhibit Consistent Functional Phenotypes Across Multiple Gene Edited Lines Despite Transcriptomic and Splicing Discrepancies.

Smith AST, Chun C, Hesson J, Mathieu J, Valdmanis PN, Mack DL, Choi BO, Kim DH, Bothwell M
Frontiers in cell and developmental biology 2021;9:728707

SARS-CoV-2 Disrupts Proximal Elements in the JAK-STAT Pathway.

Chen DY, Khan N, Close BJ, Goel RK, Blum B, Tavares AH, Kenney D, Conway HL, Ewoldt JK, Chitalia VC, Crossland NA, Chen CS, Kotton DN, Baker SC, Fuchs SY, Connor JH, Douam F, Emili A, Saeed M
Journal of virology 2021 Sep 9;95(19):e0086221

Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA.

Key J, Torres-Odio S, Bach NC, Gispert S, Koepf G, Reichlmeir M, West AP, Prokisch H, Freisinger P, Newman WG, Shalev S, Sieber SA, Wittig I, Auburger G
Cells 2021 Nov 29;10(12)

Molecular mechanisms regulating lysophosphatidylcholine acyltransferase 1 (LPCAT1) in human pregnancy.

Purandare N, Minchella P, Somayajulu M, Kramer KJ, Zhou J, Adekoya N, Welch RA, Grossman LI, Aras S, Recanati MA
Placenta 2021 Mar;106:40-48

TDP-43 and PINK1 mediate CHCHD10(S59L) mutation-induced defects in Drosophila and in vitro.

Baek M, Choe YJ, Bannwarth S, Kim J, Maitra S, Dorn GW 2nd, Taylor JP, Paquis-Flucklinger V, Kim NC
Nature communications 2021 Mar 26;12(1):1924

SARS-CoV-2 desensitizes host cells to interferon through inhibition of the JAK-STAT pathway.

Chen DY, Khan N, Close BJ, Goel RK, Blum B, Tavares AH, Kenney D, Conway HL, Ewoldt JK, Kapell S, Chitalia VC, Crossland NA, Chen CS, Kotton DN, Baker SC, Connor JH, Douam F, Emili A, Saeed M
bioRxiv : the preprint server for biology 2020 Oct 28;

Decreased membrane cholesterol in liver mitochondria of the point mutation mouse model of juvenile Niemann-Pick C1, Npc1(nmf164).

Erickson RP, Aras S, Purandare N, Hüttemann M, Liu J, Dragotto J, Fiorenza MT, Grossman LI
Mitochondrion 2020 Mar;51:15-21

Loss of CHCHD2 and CHCHD10 activates OMA1 peptidase to disrupt mitochondrial cristae phenocopying patient mutations.

Liu YT, Huang X, Nguyen D, Shammas MK, Wu BP, Dombi E, Springer DA, Poulton J, Sekine S, Narendra DP
Human molecular genetics 2020 Jun 3;29(9):1547-1567

Mitochondrial Nuclear Retrograde Regulator 1 (MNRR1) rescues the cellular phenotype of MELAS by inducing homeostatic mechanisms.

Aras S, Purandare N, Gladyck S, Somayajulu-Nitu M, Zhang K, Wallace DC, Grossman LI
Proceedings of the National Academy of Sciences of the United States of America 2020 Dec 15;117(50):32056-32065

Mitochondrial autoimmunity and MNRR1 in breast carcinogenesis.

Aras S, Maroun MC, Song Y, Bandyopadhyay S, Stark A, Yang ZQ, Long MP, Grossman LI, Fernández-Madrid F
BMC cancer 2019 May 2;19(1):411

CHCHD10 is involved in the development of Parkinson's disease caused by CHCHD2 loss-of-function mutation p.T61I.

Mao C, Wang H, Luo H, Zhang S, Xu H, Zhang S, Rosenblum J, Wang Z, Zhang Q, Tang M, Shepard MJ, Wang X, Wang Y, Zhuang Z, Shi C, Xu Y
Neurobiology of aging 2019 Mar;75:38-41

PDGFRβ translocates to the nucleus and regulates chromatin remodeling via TATA element-modifying factor 1.

Papadopoulos N, Lennartsson J, Heldin CH
The Journal of cell biology 2018 May 7;217(5):1701-1717

In vitro and in vivo studies of the ALS-FTLD protein CHCHD10 reveal novel mitochondrial topology and protein interactions.

Burstein SR, Valsecchi F, Kawamata H, Bourens M, Zeng R, Zuberi A, Milner TA, Cloonan SM, Lutz C, Barrientos A, Manfredi G
Human molecular genetics 2018 Jan 1;27(1):160-177

The cellular stress proteins CHCHD10 and MNRR1 (CHCHD2): Partners in mitochondrial and nuclear function and dysfunction.

Purandare N, Somayajulu M, Hüttemann M, Grossman LI, Aras S
The Journal of biological chemistry 2018 Apr 27;293(17):6517-6529

Reversal of Phenotypic Abnormalities by CRISPR/Cas9-Mediated Gene Correction in Huntington Disease Patient-Derived Induced Pluripotent Stem Cells.

Xu X, Tay Y, Sim B, Yoon SI, Huang Y, Ooi J, Utami KH, Ziaei A, Ng B, Radulescu C, Low D, Ng AYJ, Loh M, Venkatesh B, Ginhoux F, Augustine GJ, Pouladi MA
Stem cell reports 2017 Mar 14;8(3):619-633

Loss of Parkinson's disease-associated protein CHCHD2 affects mitochondrial crista structure and destabilizes cytochrome c.

Meng H, Yamashita C, Shiba-Fukushima K, Inoshita T, Funayama M, Sato S, Hatta T, Natsume T, Umitsu M, Takagi J, Imai Y, Hattori N
Nature communications 2017 Jun 7;8:15500

Quantitative Proteomic Analysis of Mosquito C6/36 Cells Reveals Host Proteins Involved in Zika Virus Infection.

Xin QL, Deng CL, Chen X, Wang J, Wang SB, Wang W, Deng F, Zhang B, Xiao G, Zhang LK
Journal of virology 2017 Jun 15;91(12)

Abl2 kinase phosphorylates Bi-organellar regulator MNRR1 in mitochondria, stimulating respiration.

Aras S, Arrabi H, Purandare N, Hüttemann M, Kamholz J, Züchner S, Grossman LI
Biochimica et biophysica acta. Molecular cell research 2017 Feb;1864(2):440-448

Analysis of EV71 infection progression using triple-SILAC-based proteomics approach.

Li HY, Zhang LK, Zhu XJ, Shang J, Chen X, Zhu Y, Guo L
Proteomics 2015 Nov;15(21):3629-43

CHCHD2 mutations in autosomal dominant late-onset Parkinson's disease: a genome-wide linkage and sequencing study.

Funayama M, Ohe K, Amo T, Furuya N, Yamaguchi J, Saiki S, Li Y, Ogaki K, Ando M, Yoshino H, Tomiyama H, Nishioka K, Hasegawa K, Saiki H, Satake W, Mogushi K, Sasaki R, Kokubo Y, Kuzuhara S, Toda T, Mizuno Y, Uchiyama Y, Ohno K, Hattori N
The Lancet. Neurology 2015 Mar;14(3):274-82

MNRR1 (formerly CHCHD2) is a bi-organellar regulator of mitochondrial metabolism.

Aras S, Bai M, Lee I, Springett R, Hüttemann M, Grossman LI
Mitochondrion 2015 Jan;20:43-51

Mitochondrial targeting sequence variants of the CHCHD2 gene are a risk for Lewy body disorders.

Ogaki K, Koga S, Heckman MG, Fiesel FC, Ando M, Labbé C, Lorenzo-Betancor O, Moussaud-Lamodière EL, Soto-Ortolaza AI, Walton RL, Strongosky AJ, Uitti RJ, McCarthy A, Lynch T, Siuda J, Opala G, Rudzinska M, Krygowska-Wajs A, Barcikowska M, Czyzewski K, Puschmann A, Nishioka K, Funayama M, Hattori N, Parisi JE, Petersen RC, Graff-Radford NR, Boeve BF, Springer W, Wszolek ZK, Dickson DW, Ross OA
Neurology 2015 Dec 8;85(23):2016-25

Oxygen-dependent expression of cytochrome c oxidase subunit 4-2 gene expression is mediated by transcription factors RBPJ, CXXC5 and CHCHD2.

Aras S, Pak O, Sommer N, Finley R Jr, Hüttemann M, Weissmann N, Grossman LI
Nucleic acids research 2013 Feb 1;41(4):2255-66

Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells.

Feyeux M, Bourgois-Rocha F, Redfern A, Giles P, Lefort N, Aubert S, Bonnefond C, Bugi A, Ruiz M, Deglon N, Jones L, Peschanski M, Allen ND, Perrier AL
Human molecular genetics 2012 Sep 1;21(17):3883-95

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Supportive validation

Submitted by: Proteintech Group (provider)
Main image
Experimental details: HT-1080 cells were subjected to SDS PAGE followed by western blot with 19424-1-AP(CHCHD2 antibody) at dilution of 1:200
Sample type: cell line

Supportive validation

Submitted by: Proteintech Group (provider)
Main image
Experimental details: Immunofluorescent analysis of HepG2 cells, using CHCHD2 antibody 19424-1-AP at 1:25 dilution and Rhodamine-labeled goat anti-rabbit IgG (red). Blue pseudocolor = DAPI (fluorescent DNA dye).
Sample type: cell line