AB110329

Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker

Name: Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329) | Abcam
Brand: Abcam Limited
SKU: AB110329
Price: 400 USD
Availability: InStock
Rating: 5 (3 reviews)

Lab Essentials
What is this?

(3 Reviews)

(63 Publications)

Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329) is a mouse monoclonal antibody detecting Mitofilin in Western Blot, Flow Cytometry, IHC-P, ICC/IF. Suitable for Cow, Human, Mouse, Rat.

- Over 50 publications

View Alternative Names

HMP, MIC60, MINOS2, PIG4, PIG52, IMMT, MICOS complex subunit MIC60, Cell proliferation-inducing gene 4/52 protein, Mitochondrial inner membrane protein, Mitofilin, p87/89

6 Images

PubMed

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Clinical findings for patients 1 & 2 suggest human neonatal onset mitochondrial encephalopathy with recurrent bouts of liver disease.

All lanes:

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329)

Lane 1:

Control neonatal mitochondria from skin fibroblasts

Lane 2:

Patient 2 (male) mitochondria from skin fibroblasts

Lane 3:

Patient 1 (female) mitochondria from skin fibroblasts

Predicted band size: 84 kDa

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Image from Guarani V et al., Elife. 2016;5:e17163. Fig 5(A).; doi: 10.7554/eLife.17163. Reproduced under the Creative Commons license http://creativecommons.org/licenses/by/4.0/

Immunocytochemistry/ Immunofluorescence - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

ICC/IF

Unknown

Immunocytochemistry/ Immunofluorescence - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Immunocytochemistry analysis using ab110329 at 5μg/ml staining Mitofilin in Cultured Human fibroblasts. (Fixed and permeabilized) followed by a fluorescent goat-anti-mouse IgG.

Flow Cytometry - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Flow Cyt

Unknown

Flow Cytometry - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Flow cytometric analysis using ab110329 at 1µg/ml staining Mitofilin in HL60 cells (blue). Isotype control antibody (red).

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

IHC-P

Unknown

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Immunohistological analysis using ab110329 at 1/100 dilution staining Mitofilin in Human cerebellum tissue (Formalin-fixed, Paraffin-embedded).

Unknown

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Extra bands in the mouse sample (lane 4) are due to the reaction of the IgG-specific goat anti-mouse secondary antibody with residual mouse blood in the heart tissue, as it is very difficult to entirely remove the blood from these small organs.

All lanes:

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329) at 2 µg/mL

Lane 1:

Isolated mitochondria from Human heart at 5 µg

Lane 2:

Isolated mitochondria from Bovine heart at 1 µg

Lane 3:

Isolated mitochondria from Rat heart at 10 µg

Lane 4:

Isolated mitochondria from Mouse heart at 10 µg

Lane 5:

HepG2 cell lysate at 20 µg

Predicted band size: 84 kDa

false

CiteAb

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Mitofilin western blot using anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker ab110329. Publication image and figure legend from Riley, L. G., Menezes, M. J., et al., 2013, Orphanet J Rare Dis, PubMed 24344687.

ab110329 was used in this publication in western blot. This may not be the same as the application(s) guaranteed by Abcam. For a full list of applications guaranteed by Abcam for ab110329 please see the product overview.

RC complex levels are dysregulated on myogenesis in patient muscle cells. Control (C) MyoD-forced myotubes (Myo) and muscle (Musc) have higher RC complex levels than fibroblasts (Fib) while patient (P2) MyoD-forced myotubes and muscle are deficient in Complex I (CoI NDUFB8), and IV (CoIV II).

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Key facts

Host species

Mouse

Clonality

Monoclonal

Clone number

2E4AD5

Isotype

IgG1

Light chain type

kappa

Carrier free

Reacts with

Mouse, Rat, Cow, Human

Applications

IHC-P, Flow Cyt, ICC/IF, WB

applications

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "FlowCyt" : {"fullname" : "Flow Cytometry", "shortname":"Flow Cyt"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "FlowCyt-species-checked": "testedAndGuaranteed", "FlowCyt-species-dilution-info": "1 µg/mL", "FlowCyt-species-notes": "<a href='/en-us/products/primary-antibodies/mouse-igg1-kappa-monoclonal-15-6e10a7-isotype-control-ab170190'>ab170190</a> - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "2 µg/mL", "WB-species-notes": "", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "1-5 µg/mL", "ICCIF-species-notes": "", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/100", "IHCP-species-notes": " Perform heat-mediated antigen retrieval via the microwave method before commencing with IHC staining protocol." }, "Mouse": { "FlowCyt-species-checked": "guaranteed", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "2 µg/mL", "WB-species-notes": "", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Rat": { "FlowCyt-species-checked": "guaranteed", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "2 µg/mL", "WB-species-notes": "", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Cow": { "FlowCyt-species-checked": "guaranteed", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "2 µg/mL", "WB-species-notes": "", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" } } }

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Product details

What is this antibody validated in?
Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (ab110329) is a mouse monoclonal antibody and is validated for use in Western Blot (WB), Flow Cytometry (Flow Cyt), Immunohistochemistry (IHC-P), Immunocytochemistry/immunofluorescence (ICC/IF) in Cow, Human, Mouse, Rat samples.

What is the molecular weight of Mitofilin?
Anti-Mitofilin [2E4AD5] - Mitochondrial Marker (ab110329) specifically detects a band for Mitofilin (UniProt: Q16891) at a molecular weight of 84kDa.

Trusted by the scientific community
Anti-Mitofilin [2E4AD5] - Mitochondrial Marker (ab110329) was first used in a scientific publication in 2011 and has been cited over 50 times in peer-reviewed journals.

Other related products
We have a range of other formats of antibody clone [2E4AD5] also available for your convenience: ab110329, Alexa Fluor® 488 - ab198028, Alexa Fluor® 647 - ab198036, HRP - ab198037

Want a custom formulation?
This antibody clone is manufactured by Abcam. If you require a custom buffer formulation or conjugation for your experiments, please contact orders@abcam.com

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Properties and storage information

Form

Liquid

Purification notes

ab110329 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation.Purity: >95% by SDS-PAGE.

Storage buffer

pH: 7.5 Preservative: 0.02% Sodium azide Constituents: HEPES buffered saline

Shipped at conditions

Blue Ice

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

+4°C

Storage information

Do Not Freeze

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Supplementary information

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

Mitofilin also known as mitofilin or MIM is a protein with a mass of approximately 88 kDa. This protein is integral to the inner mitochondrial membrane. Mitofilin is found in various tissues with high expression in the heart and skeletal muscle indicating its significant role in energy-demanding tissues. It is the central component of the mitochondrial inner membrane organizing system (MINOS) which plays a fundamental role in maintaining the mitochondrial architecture.

Biological function summary

Within the mitochondrial environment mitofilin contributes to maintaining cristae morphology which is essential for optimal mitochondrial respiration and energy production. As part of the MINOS complex mitofilin interacts with other proteins such as Mic60 and Mic10 stabilizing the structure of crista junctions. This process ensures efficient electron transport chain function ultimately supporting ATP synthesis. Without effective shaping of the cristae cellular energy metabolism deteriorates.

Pathways

Research has highlighted the importance of mitofilin in apoptosis and bioenergetics pathways. It interacts with proteins like OPA1 a dynamin-related GTPase important for mitochondrial fusion and energy metabolism. Additionally mitofilin influences the release of cytochrome c an important step in the initiation of apoptosis. Proper functioning of these pathways is critical for cellular balance and survival showing mitofilin's broad impact on cellular processes.

Mitofilin has links to mitochondrial myopathies and cardiomyopathies. These disorders stem from impaired mitochondrial function significantly affecting muscle tissues and heart performance. Disruption in mitofilin expression or function can lead to compromised energy production contributing to these conditions. Furthermore mitofilin has associations with proteins like Drp1 involved in mitochondrial dynamics and this relationship can influence the progression and severity of mitochondrial-related diseases.

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Product protocols

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

Visit the General protocols
Visit the Troubleshooting

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Target data

Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane (PubMed : 22114354, PubMed : 25781180, PubMed : 32567732, PubMed : 33130824). Plays an important role in the maintenance of the MICOS complex stability and the mitochondrial cristae morphology (PubMed : 22114354, PubMed : 25781180, PubMed : 32567732, PubMed : 33130824).

See full target information IMMT

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Publications (63)

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

Journal of molecular neuroscience : MN 75:131 PubMed41034456

2025

Spinocerebellar Ataxia Type 1 (SCA1) Cell Models Display Widespread Mitochondrial and Extra-Nuclear Alterations.

Applications

Unspecified application

Species

Unspecified reactive species

Dane Ford-Roshon,Madison Dudek,Ada Glynn,Abigale Glasman,Jaden York,Emily Lawrence,Donna Nguyen,Lindsay Shinn,Georgia Berry,Lily Kendall,Jennifer Bonner,Austin Ferro,Sarita Lagalwar

Cell reports. Medicine 6:102116 PubMed40328250

2025

Muscle metabolic resilience and enhanced exercise adaptation by Esr1-induced remodeling of mitochondrial cristae-nucleoid architecture in males.

Applications

Unspecified application

Species

Mouse

Zhenqi Zhou,Timothy M Moore,Alexander R Strumwasser,Vicent Ribas,Hirotaka Iwasaki,Noelle Morrow,Alice Ma,Peter H Tran,Jonathan Wanagat,Thomas Q de Aguiar Vallim,Bethan Clifford,Zhengyi Zhang,Tamer Sallam,Brian W Parks,Karen Reue,Orian Shirihai,Rebeca Acin-Perez,Marco Morselli,Matteo Pellegrini,Sushil K Mahata,Frode Norheim,Mingqi Zhou,Marcus M Seldin,Aldons J Lusis,Cathy C Lee,Mark O Goodarzi,Jerome I Rotter,Joshua R Hansen,Ben Drucker,Tyler J Sagendorf,Joshua N Adkins,James A Sanford,Francesco J DeMayo,Sylvia C Hewitt,Kenneth S Korach,Andrea L Hevener

iScience 27:111467 PubMed39720525

2024

SLP2 and MIC13 synergistically coordinate MICOS assembly and crista junction formation.

Applications

Unspecified application

Species

Unspecified reactive species

Ritam Naha,Rebecca Strohm,Yulia Schaumkessel,Jennifer Urbach,Ilka Wittig,Andreas S Reichert,Arun Kumar Kondadi,Ruchika Anand

FASEB journal : official publication of the Federation of American Societies for Experimental Biology 38:e70223 PubMed39625361

2024

Purification of mitochondria from skeletal muscle tissue for transcriptomic analyses reveals localization of nuclear-encoded noncoding RNAs.

Applications

Unspecified application

Species

Unspecified reactive species

Jessica Silver,Adam J Trewin,Stella Loke,Larry Croft,Mark Ziemann,Megan Soria,Hayley Dillon,Søren Nielsen,Séverine Lamon,Glenn D Wadley

Advanced science (Weinheim, Baden-Wurttemberg, Germany) 11:e2406760 PubMed39431455

2024

The MICOS Complex Subunit Mic60 is Hijacked by Intracellular Bacteria to Manipulate Mitochondrial Dynamics and Promote Bacterial Pathogenicity.

Applications

Unspecified application

Species

Unspecified reactive species

Changyong Cheng,Mianmian Chen,Jing Sun,Jiali Xu,Simin Deng,Jing Xia,Yue Han,Xian Zhang,Jing Wang,Lei Lei,Ruidong Zhai,Qin Wu,Weihuan Fang,Houhui Song

Methods in molecular biology (Clifton, N.J.) 2782:113-122 PubMed38622396

2024

Endothelial Mitochondria-Associated Membranes (MAMs) Isolation by Percoll Step Gradients.

Applications

Unspecified application

Species

Unspecified reactive species

Margaret Baldini,Cheng Zhang,Jun Yu

Cardiovascular diabetology 23:96 PubMed38486199

2024

MCT4-dependent lactate transport: a novel mechanism for cardiac energy metabolism injury and inflammation in type 2 diabetes mellitus.

Applications

Unspecified application

Species

Unspecified reactive species

Xiu Mei Ma,Kang Geng,Peng Wang,Zongzhe Jiang,Betty Yuen-Kwan Law,Yong Xu

Cell reports 42:113544 PubMed38060381

2023

A mitochondrial inside-out iron-calcium signal reveals drug targets for Parkinson's disease.

Applications

Unspecified application

Species

Unspecified reactive species

Vinita Bharat,Aarooran S Durairaj,Roeland Vanhauwaert,Li Li,Colin M Muir,Sujyoti Chandra,Chulhwan S Kwak,Yann Le Guen,Pawan Nandakishore,Chung-Han Hsieh,Stefano E Rensi,Russ B Altman,Michael D Greicius,Liang Feng,Xinnan Wang

Aging cell 22:e14009 PubMed37960952

2023

3D reconstruction of murine mitochondria reveals changes in structure during aging linked to the MICOS complex.

Applications

Unspecified application

Species

Unspecified reactive species

Zer Vue,Edgar Garza-Lopez,Kit Neikirk,Prasanna Katti,Larry Vang,Heather Beasley,Jianqiang Shao,Andrea G Marshall,Amber Crabtree,Alexandria C Murphy,Brenita C Jenkins,Praveena Prasad,Chantell Evans,Brittany Taylor,Margaret Mungai,Mason Killion,Dominique Stephens,Trace A Christensen,Jacob Lam,Benjamin Rodriguez,Mark A Phillips,Nastaran Daneshgar,Ho-Jin Koh,Alice Koh,Jamaine Davis,Nina Devine,Mohammad Saleem,Estevão Scudese,Kenneth Ryan Arnold,Valeria Vanessa Chavarin,Ryan Daniel Robinson,Moumita Chakraborty,Jennifer A Gaddy,Mariya T Sweetwyne,Genesis Wilson,Elma Zaganjor,James Kezos,Cristiana Dondi,Anilkumar K Reddy,Brian Glancy,Annet Kirabo,Anita M Quintana,Dao-Fu Dai,Karen Ocorr,Sandra A Murray,Steven M Damo,Vernat Exil,Blake Riggs,Bret C Mobley,Jose A Gomez,Melanie R McReynolds,Antentor Hinton

Molecular cell 83:3188-3204.e7 PubMed37683611

2023

Damaged mitochondria recruit the effector NEMO to activate NF-κB signaling.

Applications

Unspecified application

Species

Unspecified reactive species

Olivia Harding,Elisabeth Holzer,Julia F Riley,Sascha Martens,Erika L F Holzbaur

View all publications

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Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.

For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

For licensing inquiries, please contact partnerships@abcam.com

Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Immunocytochemistry/ Immunofluorescence - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Flow Cytometry - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Western blot - Anti-Mitofilin antibody [2E4AD5] - Mitochondrial Marker (AB110329)

Key facts

Host species

Clonality

Clone number

Isotype

Light chain type

Carrier free

Reacts with

Applications

Reactivity data

Product details

Properties and storage information

Form

Purification notes

Storage buffer

Shipped at conditions

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Storage information

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Publications (63)

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