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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...
Mitochondrial Precursor Proteins01:39

Mitochondrial Precursor Proteins

Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
Most of the mitochondrial precursors...
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...
The ADP/ATP Carrier Protein01:42

The ADP/ATP Carrier Protein

ADP/ATP carrier or AAC protein is the most abundant carrier protein in the inner mitochondrial membrane. It transports large quantities of ADP and ATP, equivalent to the average human body weight, every day. Among other transporters, ACC protein is one of the best-studied members of the mitochondrial carrier protein family. The ADP/ATP carrier protein comprises two transmembrane helices connected to a loop and a single alpha-helix on the matrix side. It switches between two conformational...
Mitochondrial Protein Sorting01:39

Mitochondrial Protein Sorting

Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
Most of these mitochondrial proteins are encoded by the nucleus and imported to the mitochondria as unfolded or loosely folded precursors. Mitochondrial precursors...
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:
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A Screenable In Vivo Assay for Mitochondrial Modulators Using Transgenic Bioluminescent Caenorhabditis elegans
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C. elegans ATAD-3 is essential for mitochondrial activity and development.

Michael Hoffmann1, Nadège Bellance, Rodrigue Rossignol

  • 1Department of General Pediatrics, University Children's Hospital, Heinrich-Heine-University, Düsseldorf, Germany.

Plos One
|November 6, 2009
PubMed
Summary

The ATAD-3 protein is essential for mitochondrial function and development in C. elegans. Depletion of ATAD-3 leads to developmental arrest linked to reduced mitochondrial activity and metabolic dysfunction.

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Area of Science:

  • Mitochondrial biology
  • Developmental biology
  • Molecular genetics

Background:

  • ATAD3 is a mitochondrial protein implicated in nucleoid organization.
  • The precise function of ATAD3 remains largely unresolved in mammals.

Purpose of the Study:

  • Characterize the ATAD3 homologue (ATAD-3) in Caenorhabditis elegans (C. elegans).
  • Investigate the role of ATAD-3 in mitochondrial function and overall development.

Main Methods:

  • RNA-mediated interference (RNAi) to deplete ATAD-3 in C. elegans.
  • Analysis of developmental phenotypes, including larval arrest and lethality.
  • Assessment of mitochondrial physiology, including organellar structure, complex I, and citrate synthase activities.
  • Evaluation of metabolic parameters like intestinal fat storage and lysosomal content.

Main Results:

  • ATAD-3 is highly conserved across species.
  • RNAi-mediated depletion of ATAD-3 resulted in severe developmental defects (larval arrest, gonadal dysfunction, embryonic lethality).
  • Mitochondrial structure was disturbed, but biogenesis and function (complex I, citrate synthase) were stage-dependent, with low activity in L1 larvae.
  • ATAD-3 depletion led to reduced intestinal fat storage and lysosomal content, indicating a role in metabolic activity.
  • Animals arrested at developmental stages correlating with low mitochondrial activity.

Conclusions:

  • ATAD-3 is crucial for C. elegans development in vivo.
  • ATAD-3 plays a significant role in upregulating mitochondrial activity during larval development.