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Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
Sorting of outer membrane proteins:
Mitochondrial outer membrane proteins are of two types: the transmembrane, beta-barrel porins, and the membrane-anchored, alpha-helical proteins. Beta-barrel porin precursors are translocated by the TOM complex and inserted into the outer mitochondrial membrane by the SAM complex. In contrast,...
Electron Transport Chain: Complex I and II01:46

Electron Transport Chain: Complex I and II

The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...
The Inner Mitochondrial Membrane01:28

The Inner Mitochondrial Membrane

The inner mitochondrial membrane is the primary site of ATP synthesis. The inner membrane domain that forms a smooth layer adjacent to the outer membrane is called the inner boundary membrane. This domain contains membrane transporters that drive metabolites in and out of the mitochondria.  In contrast, the inner membrane network that invaginates into the matrix space is called the cristae membrane. This domain accounts for principle mitochondrial function as it accommodates the protein...
Mitochondria01:37

Mitochondria

Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
Mitochondrial Membranes01:45

Mitochondrial Membranes

A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
Mitochondrial Membranes01:45

Mitochondrial Membranes

A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...

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Enhanced Response to Radiopharmaceutical Therapy in Preclinical Tumor Models with XRD-0394, a Dual Inhibitor of ATM Kinase and DNA-PKcs.

Radiation research·2026
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ATM interaction with GRP94 modulates oncogenic receptor expression and signaling and microglial activation.

Proceedings of the National Academy of Sciences of the United States of America·2025
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Author Correction: Cancer-associated DDX3X mutations drive stress granule assembly and impair global translation.

Scientific reports·2024
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A Novel Dual ATM/DNA-PK Inhibitor, XRD-0394, Potently Radiosensitizes and Potentiates PARP and Topoisomerase I Inhibitors.

Molecular cancer therapeutics·2024
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Participation of ATM, SMG1, and DDX5 in a DNA Damage-Induced Alternative Splicing Pathway.

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ATM Regulation of the Cohesin Complex Is Required for Repression of DNA Replication and Transcription in the Vicinity of DNA Double-Strand Breaks.

Molecular cancer research : MCR·2022
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Related Experiment Video

Updated: May 22, 2026

Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome
07:56

Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome

Published on: November 30, 2022

A new role for ATM: regulating mitochondrial function and mitophagy.

Yasmine A Valentin-Vega1, Michael B Kastan

  • 1Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.

Autophagy
|May 24, 2012
PubMed
Summary

Ataxia telangiectasia (A-T) is linked to DNA damage response defects. In A-T mice, impaired mitophagy causes mitochondrial issues, but blocking autophagy delays tumor growth, suggesting ATM regulates mitochondrial homeostasis.

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Sensitive Measurement of Mitophagy by Flow Cytometry Using the pH-dependent Fluorescent Reporter mt-Keima
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Sensitive Measurement of Mitophagy by Flow Cytometry Using the pH-dependent Fluorescent Reporter mt-Keima

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Detection of Mitophagy in Caenorhabditis elegans and Mammalian Cells Using Organelle-Specific Dyes

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Last Updated: May 22, 2026

Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome
07:56

Visualizing Mitophagy with Fluorescent Dyes for Mitochondria and Lysosome

Published on: November 30, 2022

Sensitive Measurement of Mitophagy by Flow Cytometry Using the pH-dependent Fluorescent Reporter mt-Keima
09:13

Sensitive Measurement of Mitophagy by Flow Cytometry Using the pH-dependent Fluorescent Reporter mt-Keima

Published on: August 12, 2018

Detection of Mitophagy in Caenorhabditis elegans and Mammalian Cells Using Organelle-Specific Dyes
11:59

Detection of Mitophagy in Caenorhabditis elegans and Mammalian Cells Using Organelle-Specific Dyes

Published on: May 19, 2023

Area of Science:

  • Cell Biology
  • Genetics
  • Immunology

Background:

  • Ataxia telangiectasia (A-T) is a genetic disorder linked to mutations in the ATM gene, which is crucial for DNA damage response (DDR).
  • ATM's role in T-cell lymphomagenesis and other pathologies in A-T patients is well-established, primarily through its function in DDR.
  • Mitochondrial dysfunction has been observed in A-T, but its direct link to ATM deficiency and disease progression is less understood.

Purpose of the Study:

  • To investigate the role of ATM in mitochondrial homeostasis and mitophagy in the context of T-cell lymphomagenesis.
  • To determine if modulating autophagy can impact tumorigenesis in ATM-deficient models.
  • To elucidate the relationship between ATM, mitochondrial function, and DNA damage response.

Main Methods:

  • Analysis of Atm-deficient thymocytes in a mouse model.
  • Assessment of mitochondrial homeostasis and mitophagy engagement in these cells.
  • Evaluation of the impact of allelic loss of the autophagy gene Becn1 on tumorigenesis in Atm-null mice.

Main Results:

  • Atm deficiency in thymocytes leads to altered mitochondrial homeostasis, associated with abnormal mitophagy.
  • Allelic loss of Becn1 delays tumor development in Atm-null mice.
  • The delay in tumorigenesis appears to be due to the reversal of mitochondrial abnormalities, not an improvement in the DNA damage response (DDR) pathway.

Conclusions:

  • ATM plays a critical role in maintaining mitochondrial homeostasis, potentially through the regulation of mitophagy.
  • Autophagy modulation, specifically Becn1 loss, can counteract mitochondrial defects and delay tumorigenesis in ATM-deficient contexts.
  • These findings suggest that targeting mitochondrial pathways could be a therapeutic strategy for A-T-related cancers.