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AB171978

Anti-TIMMDC1/C3orf1 antibody [EPR11807]

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(4 Publications)

Rabbit Recombinant Monoclonal TIMMDC1/C3orf1 antibody. Suitable for IP, WB, ICC/IF and reacts with Human samples. Cited in 4 publications.

View Alternative Names

C3orf1, UNQ247/PRO284, TIMMDC1, Protein M5-14, Translocase of inner mitochondrial membrane domain-containing protein 1, TIMM domain containing-protein 1

4 Images
Immunocytochemistry/ Immunofluorescence - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)
  • ICC/IF

Supplier Data

Immunocytochemistry/ Immunofluorescence - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)

Immunofluorescent analysis of HepG2 cells labeling TIMMDC1/C3orf1 with ab171978 at 1/100 dilution (red). DAPI nuclear staining (blue).

Immunoprecipitation - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)
  • IP

Supplier Data

Immunoprecipitation - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)

Western blot analysis on immunoprecipitation pellet from A375 cell lysate labeling TIMMDC1/C3orf1 with ab171978 at 1/10 dilution.

All lanes:

Immunoprecipitation - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (ab171978)

Predicted band size: 32 kDa

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Western blot - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)
  • WB

Supplier Data

Western blot - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)

All lanes:

Western blot - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (ab171978) at 1/10000 dilution

Lane 1:

THP1 cell lysate at 10 µg

Lane 2:

U937 (Human histiocytic lymphoma cell line) cell lysate at 10 µg

Lane 3:

A375 cell lysate at 10 µg

Lane 4:

HepG2 cell lysate at 10 µg

Lane 5:

Fetal heart tissue lysate at 10 µg

Predicted band size: 32 kDa

false

Western blot - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)
  • WB

CiteAb

Western blot - Anti-TIMMDC1/C3orf1 antibody [EPR11807] (AB171978)

TIMMDC1/C3orf1 western blot using anti-TIMMDC1/C3orf1 antibody [EPR11807] ab171978. Publication image and figure legend from Kremer, L. S., Bader, D. M., et al., 2017, Nat Commun, PubMed 28604674.

ab171978 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 ab171978 please see the product overview.

RNA aberrant expression detection and validation.(a) Aberrantly expressed genes (Hochberg corrected P value<0.05 and |Z-score|>3) for each patient fibroblasts. (b) Gene-wise RNA expression volcano plot of nominal P values (-log10P value) against Z-scores of the patient #35791 compared against all other fibroblasts. Z-scores with absolute value >5 are plotted at ±5, respectively. (c) Same as (b) for patient #73804. (d) Sample-wise RNA expression is ranked for the genes TIMMDC1 (top) and MGST1 (bottom). Samples with aberrant expression for the corresponding gene are highlighted in red (#35791, #66744, and #73804). (e) Gene-wise comparison of RNA and protein fold changes of patient #35791 compared to the average across the fibroblast cell lines of all other patients. Subunits of the mitochondrial respiratory chain complex I are highlighted (red squares). Reliably detected proteins that were not detected in this sample are shown separately with their corresponding RNA fold changes (points below solid horizontal line). (f) Western blot of TIMMDC1, NDUFA13, NDUFB3 and NDUFB8 protein in three fibroblast cell lines without (#62346, #91324, NHDF) and three with a variant in TIMMDC1 (#35791, #66744 and #96687), and fibroblasts re-expressing TIMMDC1 ('-T') (#35791-T, #66744-T and #96687-T). UQCRC2 was used as loading control. CI, complex I subunit; CIII, complex III subunit; MW, molecular weight. (g) Blue native PAGE blot of the control fibroblasts re-expressing TIMMDC1 (NHDF-T), the control fibroblasts (NHDF), patient fibroblasts (#96687) and patient fibroblast re-expressing TIMMDC1 (#96687-T). Immunodecoration for complex I and complex III was performed using NDUFB8 and UQCRC2 antibodies, respectively. CI, complex I subunit; CIII, complex III subunit.

false

  • Carrier free

    Anti-TIMMDC1/C3orf1 antibody [EPR11807] - BSA and Azide free

Key facts

Host species

Rabbit

Clonality

Monoclonal

Clone number

EPR11807

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

IP, ICC/IF, WB

applications

Immunogen

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

Reactivity data

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Product details

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

What are the advantages of a recombinant monoclonal antibody?
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.

Properties and storage information

Form
Liquid
Purity
Tissue culture supernatant
Storage buffer
pH: 7.2 - 7.4 Preservative: 0.01% Sodium azide Constituents: PBS, 50% Tissue culture supernatant, 40% Glycerol (glycerin, glycerine), 0.05% BSA
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

Supplementary information

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

TIMMDC1 also known as C3orf1 is a mitochondrial protein with a mass of approximately 34 kDa. It plays a mechanical role in the assembly of mitochondrial respiratory chain complex I. It becomes integrated into the inner mitochondrial membrane where it acts as an important assembly factor. TIMMDC1 is expressed ubiquitously across various human tissues with relatively higher levels in tissues with high energy demands like the heart and skeletal muscle.
Biological function summary

TIMMDC1 is essential in maintaining cellular energy metabolism. It is a component of the mitochondrial complex I assembly pathway critical for the formation and stabilization of the entire complex I. Complex I is responsible for the initial steps of the electron transport chain important for ATP production via oxidative phosphorylation. TIMMDC1 interacts with other complex I assembly factors contributing to the efficient generation of ATP within mitochondria.

Pathways

TIMMDC1 has a significant impact on the oxidative phosphorylation pathway and is a pivotal part of the mitochondrial respiratory chain. Its activity influences cellular respiration by assisting the functional integrity of complex I which interacts closely with other oxidative phosphorylation participants such as NADH dehydrogenase (ubiquinone) and cytochrome c oxidase. TIMMDC1 plays its role alongside these proteins facilitating the production of energy that cells require for various metabolic processes.

Defects in TIMMDC1 have been linked to mitochondrial complex I deficiency a condition that can result in a spectrum of disorders such as Leigh syndrome and other encephalomyopathies. This protein's malfunction can disrupt normal energy production leading to energy deficits in cells particularly in the nervous system and muscles. Additionally disruptions in the TIMMDC1 protein interaction with other mitochondrial proteins can exacerbate disease phenotypes such as with NDUFAF3 another mitochondrial assembly factor associated with complex I deficiency.

Product protocols

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

Target data

Chaperone protein involved in the assembly of the mitochondrial NADH : ubiquinone oxidoreductase complex (complex I). Participates in constructing the membrane arm of complex I.
See full target information TIMMDC1

Publications (4)

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

The EMBO journal 41:e110476 PubMed35912435

2022

Regulation of mitochondrial proteostasis by the proton gradient.

Applications

Unspecified application

Species

Unspecified reactive species

Maria Patron,Daryna Tarasenko,Hendrik Nolte,Lara Kroczek,Mausumi Ghosh,Yohsuke Ohba,Yvonne Lasarzewski,Zeinab Alsadat Ahmadi,Alfredo Cabrera-Orefice,Akinori Eyiama,Tim Kellermann,Elena I Rugarli,Ulrich Brandt,Michael Meinecke,Thomas Langer

NPJ genomic medicine 7:9 PubMed35091571

2022

Oligonucleotide correction of an intronic TIMMDC1 variant in cells of patients with severe neurodegenerative disorder.

Applications

Unspecified application

Species

Unspecified reactive species

Raman Kumar,Mark A Corbett,Nicholas J C Smith,Daniella H Hock,Zoya Kikhtyak,Liana N Semcesen,Atsushi Morimoto,Sangmoon Lee,David A Stroud,Joseph G Gleeson,Eric A Haan,Jozef Gecz

Biochemical and biophysical research communications 508:749-755 PubMed30528236

2018

Long non-coding RNA TCF7 contributes to the growth and migration of airway smooth muscle cells in asthma through targeting TIMMDC1/Akt axis.

Applications

Unspecified application

Species

Unspecified reactive species

Maiying Fan,Jing Xu,Qiming Xiao,Fang Chen,Xiaotong Han

Nature communications 8:15824 PubMed28604674

2017

Genetic diagnosis of Mendelian disorders via RNA sequencing.

Applications

WB

Species

Human

Laura S Kremer,Daniel M Bader,Christian Mertes,Robert Kopajtich,Garwin Pichler,Arcangela Iuso,Tobias B Haack,Elisabeth Graf,Thomas Schwarzmayr,Caterina Terrile,Eliška Koňaříková,Birgit Repp,Gabi Kastenmüller,Jerzy Adamski,Peter Lichtner,Christoph Leonhardt,Benoit Funalot,Alice Donati,Valeria Tiranti,Anne Lombes,Claude Jardel,Dieter Gläser,Robert W Taylor,Daniele Ghezzi,Johannes A Mayr,Agnes Rötig,Peter Freisinger,Felix Distelmaier,Tim M Strom,Thomas Meitinger,Julien Gagneur,Holger Prokisch
View all publications

Product promise

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