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Related Concept Videos

ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased ATP...
Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
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,...
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...
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,...

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Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle
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Mitochondrial dysfunction in ataxia-telangiectasia.

Yasmine A Valentin-Vega1, Kirsteen H Maclean, Jacqueline Tait-Mulder

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

Blood
|December 7, 2011
PubMed
Summary
This summary is machine-generated.

Loss of ATM protein causes mitochondrial dysfunction and reactive oxygen species (ROS) in thymocytes, contributing to T-cell cancers. Reducing autophagy via Beclin-1 loss reversed mitochondrial issues, delaying tumor development in ATM-null mice.

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Published on: January 22, 2017

Area of Science:

  • Cell Biology
  • Genetics
  • Cancer Research

Background:

  • Ataxia-telangiectasia mutated (ATM) is crucial for DNA damage response and preventing T-cell malignancies.
  • ATM deficiency is linked to cancer development, but its precise role in cellular processes beyond DNA repair is not fully understood.

Purpose of the Study:

  • To investigate the role of ATM in mitochondrial homeostasis within thymocytes.
  • To explore the impact of ATM loss on mitochondrial function and its contribution to T-cell malignancies.
  • To determine if modulating autophagy can influence tumor development in ATM-deficient models.

Main Methods:

  • Analysis of mitochondrial function (ROS levels, respiratory capacity, mitophagy) in thymocytes from ATM-null mice.
  • Investigating the localization and activation of ATM within mitochondria.
  • Examining the effect of Beclin-1 (autophagy regulator) allelic loss on tumor development and mitochondrial parameters in ATM-null mice.

Main Results:

  • ATM loss induced intrinsic mitochondrial abnormalities in thymocytes, including elevated ROS, increased aberrant mitochondria, high respiratory capacity, and reduced mitophagy.
  • ATM protein was found in mitochondria and activated by mitochondrial dysfunction.
  • Allelic loss of Beclin-1 significantly delayed tumor development in ATM-null mice by reversing mitochondrial abnormalities, not by rescuing DNA damage signaling.

Conclusions:

  • ATM plays a direct role in maintaining mitochondrial homeostasis.
  • Mitochondrial dysfunction and increased ROS in ATM-deficient cells contribute to the cancer-prone phenotype.
  • Ataxia-telangiectasia exhibits characteristics of a mitochondrial disease, suggesting novel therapeutic avenues targeting mitochondria.