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Proteins

Mitochondrial Disulphide Relay Substrate FAM136A Safeguards IMS Proteostasis and Cellular Fitness

Strategic Insights

— 1 min read

Researchers at Ludwig-Maximilians-Universitat Munchen have discovered a crucial component in the mitochondrial intermembrane space, FAM136A, which is essential for maintaining cellular health. According to the study, FAM136A is a substrate of the mitochondrial disulphide relay, a key machinery for protein import and oxidative folding in the mitochondria. The research found that FAM136A plays a vital role in maintaining the stability of other proteins in the intermembrane space, and its deletion leads to cellular dysfunction and the aggregation of other proteins.

Key Takeaways:

  • FAM136A is a substrate of the mitochondrial disulphide relay, a critical machinery for protein import and oxidative folding in the mitochondria.
  • FAM136A introduces four disulphide bonds in two twin-CXC motifs of itself, which are essential for its import and stability in the intermembrane space.
  • The study found that FAM136A forms non-covalent homodimers as a mature protein, and its deletion curtails cellular proliferation capacity and induces the integrated stress response.
  • The research demonstrated that FAM136A safeguards intermembrane space proteostasis and cellular fitness, making it a pivotal component of the IMS proteostasis network.
  • The study provides new insights into the mechanisms of mitochondrial protein import and oxidative folding.
  • The researchers identified a transient interaction between FAM136A and MIA40, which is essential for FAM136A's import into the intermembrane space.
  • The study suggests that FAM136A plays a critical role in maintaining the stability of other proteins in the intermembrane space.

Statistics:

  • The research found that FAM136A forms four disulphide bonds in two twin-CXC motifs.
  • Studies have shown that FAM136A is essential for maintaining cellular fitness.
  • The study demonstrated that deletion of FAM136A leads to a strong induction of the integrated stress response.
  • The research found that the deletion of FAM136A also leads to the aggregation of other proteins, including HAX1 and CLPB.
  • The study suggests that FAM136A plays a critical role in maintaining the proteostasis of the intermembrane space.

Sources:

  • The mitochondrial disulphide relay substrate FAM136A safeguards IMS proteostasis and cellular fitness. Redox Biology, 2025;87:103884.
  • NewsRx. Recent Reports from Ludwig-Maximilians-Universitat Munchen Highlight Findings in Biology (The mitochondrial disulphide relay substrate FAM136A safeguards IMS proteostasis and cellular fitness). Life Science Weekly. November 4, 2025; p 4060.

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