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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.
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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.
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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,...
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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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An Integrated Approach for Microprotein Identification and Sequence Analysis
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Mitochondrion-located peptides and their pleiotropic physiological functions.

Xintong Zheng1, Mengqing Xiang1,2

  • 1State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Sun Yat-sen University, Guangzhou, China.

The FEBS Journal
|May 23, 2022
PubMed
Summary

Mitochondrion-located peptides (MLPs), translated from small open reading frames, regulate key mitochondrial functions. Their deficiency disrupts vital physiological processes, highlighting their crucial roles in health and disease.

Keywords:
Mitochondrion-located peptides (MLPs)lncRNAmicropeptidesmitochondriasORF-encoded peptides (SEPs)single transmembrane domain (STMD)small open reading frames (sORFs)

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Area of Science:

  • Biochemistry
  • Molecular Biology
  • Cell Biology

Background:

  • Advanced technologies reveal small open reading frames (sORFs) translate into micropeptides.
  • A significant subset of these micropeptides are localized to mitochondria, termed mitochondrion-located peptides (MLPs).
  • MLPs are often conserved, contain transmembrane domains, and regulate mitochondrial functions.

Purpose of the Study:

  • To investigate the roles and significance of mitochondrion-located peptides (MLPs).
  • To understand the impact of MLP deficiency on physiological processes.
  • To provide insights into mitochondrion-associated biological processes and diseases.

Main Methods:

  • Analysis of sORF translation products.
  • Identification and characterization of mitochondrion-localized peptides.
  • Assessment of MLP function and conservation across species.
  • Evaluation of physiological consequences of MLP deficiency in vitro and in vivo.

Main Results:

  • MLPs play regulatory roles in mitochondrial electron transport, reactive oxygen species (ROS) production, and metabolic homeostasis.
  • Deficiency in MLPs leads to disruptions in immunity, differentiation, and metabolism.
  • MLPs are crucial for maintaining normal physiological functions.

Conclusions:

  • MLPs are essential regulators of mitochondrial function and overall physiology.
  • Understanding MLPs offers new insights into mitochondrion-related diseases.
  • MLPs represent a significant area for future research in molecular and cellular biology.