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Related Concept Videos

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

Porin Insertion in the Outer Mitochondrial Membrane

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.
Three models describe the assembly of porins by the SAM complex and their insertion into the outer membrane. Model 1 suggests that porins are assembled outside the SAM channel as the...
Mitochondria01:37

Mitochondria

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,...
Mitochondria01:37

Mitochondria

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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Related Experiment Video

Updated: May 30, 2026

Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA
12:35

Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA

Published on: November 14, 2017

RNA processing in human mitochondria.

Maria I G Lopez Sanchez1, Tim R Mercer, Stefan M K Davies

  • 1Western Australian Institute for Medical Research and Centre for Medical Research, The University of Western Australia, Perth, WA Australia.

Cell Cycle (Georgetown, Tex.)
|August 23, 2011
PubMed
Summary
This summary is machine-generated.

Mitochondrial tRNA processing is elucidated, identifying key proteins like MRPP1 and MRPP3 for 5' end processing and ELAC2/PTCD1 for 3' end processing. MRPP1 is vital for mitochondrial function.

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Discrimintion and Mapping of the Primary and Processed Transcripts in Maize Mitochondrion Using a Circular RT-PCR-based Strategy
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Discrimintion and Mapping of the Primary and Processed Transcripts in Maize Mitochondrion Using a Circular RT-PCR-based Strategy

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High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution
10:47

High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution

Published on: May 5, 2023

Related Experiment Videos

Last Updated: May 30, 2026

Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA
12:35

Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA

Published on: November 14, 2017

Discrimintion and Mapping of the Primary and Processed Transcripts in Maize Mitochondrion Using a Circular RT-PCR-based Strategy
07:26

Discrimintion and Mapping of the Primary and Processed Transcripts in Maize Mitochondrion Using a Circular RT-PCR-based Strategy

Published on: July 29, 2019

High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution
10:47

High-Throughput Image-Based Quantification of Mitochondrial DNA Synthesis and Distribution

Published on: May 5, 2023

Area of Science:

  • Molecular Biology
  • Genetics
  • Biochemistry

Background:

  • Mammalian mitochondrial DNA (mtDNA) produces polycistronic transcripts requiring precise processing.
  • The specific mechanisms for excising mitochondrial tRNAs (mt-tRNAs) from these transcripts are largely unknown.
  • Understanding mt-tRNA processing is crucial for mitochondrial gene expression and function.

Purpose of the Study:

  • To investigate the roles of specific proteins, including ELAC2, mitochondrial RNase P proteins (MRPP1 and MRPP3), and pentatricopeptide repeat domain protein 1 (PTCD1), in mt-tRNA processing.
  • To map the precise processing sites within mitochondrial transcripts affected by these proteins.
  • To determine the functional significance of these processing events.

Main Methods:

  • Deep sequencing was employed to analyze the 5' and 3' ends of processed mitochondrial transcripts.
  • Bioinformatic analysis was used to create a detailed map of mt-tRNA processing sites.
  • Functional assays were conducted to assess the impact of protein depletion or mutations on mitochondrial function.

Main Results:

  • MRPP1 and MRPP3 were identified as key enzymes responsible for the 5' end processing of mt-tRNAs and an unconventional site in the CO1 transcript.
  • ELAC2 and PTCD1 were found to be involved in the 3' end processing of mt-tRNAs.
  • MRPP1 was demonstrated to be essential for overall transcript processing, RNA modification, translation, and mitochondrial respiration.

Conclusions:

  • This study reveals the distinct roles of specific proteins in the 5' and 3' end processing of mammalian mitochondrial transcripts.
  • The findings highlight the critical importance of accurate mt-tRNA processing for maintaining mitochondrial integrity and function.
  • MRPP1 plays a central, essential role in multiple aspects of mitochondrial gene expression and cellular respiration.