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Related Concept Videos

Mitochondrial Protein Sorting01:39

Mitochondrial Protein Sorting

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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...
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Mitochondrial Precursor Proteins01:39

Mitochondrial Precursor Proteins

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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...
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Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

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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...
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Porin Insertion in the Outer Mitochondrial Membrane01:12

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Porins are beta-barrel proteins translocated to the mitochondrial outer membrane through the TOM complex into the intermembrane space. Porin precursors bind TIM chaperones within the intermembrane space and are guided to the Sorting and Assembly Machinery complex or SAM complex on the outer mitochondrial membrane.
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Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

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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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Structure of Porins01:21

Structure of Porins

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Mitochondria, chloroplasts, and gram-negative bacteria have transmembrane, beta-barrel proteins called porins to mediate the free diffusion of ions and metabolites across the membrane. Mitochondrial porin precursors contain conserved amino acid sequences called beta signals at their C-terminal. Beta signals have a  motif of PoXGXXHyXHy (Po-Polar, X-Any amino acid, G-Glycine, Hy-LargeHydrophobic), which are crucial for precursor recognition to initiate precursor assembly. Beta-barrel...
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Measurement of Protein Import Capacity of Skeletal Muscle Mitochondria
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Long-lived mitochondrial proteins and why they exist.

Ewa Bomba-Warczak1, Jeffrey N Savas1

  • 1Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.

Trends in Cell Biology
|February 28, 2022
PubMed
Summary

Mitochondria contain long-lived proteins (LLPs) that provide structural support, challenging the idea that mitochondria are always dynamic. These mitochondrial LLPs (mt-LLPs) may maintain mitochondrial structure over a lifetime.

Keywords:
cristae ultrastructurelong-lived proteinsmitochondriamitochondrial dynamicsprotein turnoverstable structures

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Area of Science:

  • Cell Biology
  • Mitochondrial Biology
  • Protein Biochemistry

Background:

  • Intracellular long-lived proteins (LLPs) are crucial for cellular homeostasis and function.
  • Mitochondrial long-lived proteins (mt-LLPs) have been identified in inner mitochondrial membranes (IMMs).
  • Mitochondria are dynamic organelles undergoing constant remodeling via fission, fusion, biogenesis, and quality control.

Purpose of the Study:

  • To propose that a subset of mitochondrial proteins persists over extended periods.
  • To suggest that these mt-LLPs offer structural support for lifelong mitochondrial integrity.

Main Methods:

  • This is an opinion article, presenting a hypothesis based on existing literature.
  • It synthesizes current knowledge on LLPs and mitochondrial dynamics.

Main Results:

  • The existence of mt-LLPs in IMMs and cristae invaginations is reported.
  • This finding contrasts with the dynamic nature of mitochondria.

Conclusions:

  • A subset of the mitochondrial proteome consists of long-lived proteins (mt-LLPs).
  • These mt-LLPs likely provide essential structural support for maintaining mitochondrial structure throughout life.