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Related Concept Videos

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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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RNA editing is a post-transcriptional modification where a precursor mRNA (pre-mRNA) nucleotide sequence is changed by base insertion, deletion, or modification. The extent of RNA editing varies from a few hundred bases, in mitochondrial DNA of trypanosomes, to a just single base, in nuclear genes of mammals. Even a single base change in the pre-mRNA can convert a codon for one amino acid into the codon for another amino acid or a stop codon. This type of re-coding can significantly affect the...
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Chemiosmosis

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Oxidative phosphorylation is a highly efficient process that generates large amounts of adenosine triphosphate (ATP), the basic unit of energy that drives many cellular processes. Oxidative phosphorylation involves two processes— the electron transport chain and chemiosmosis.
Electron Transport Chain
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Translocation of Proteins into the Mitochondria01:19

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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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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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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.
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Updated: May 31, 2025

Genotyping Single Nucleotide Polymorphisms in the Mitochondrial Genome by Pyrosequencing
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Mitochondrial base editing: from principle, optimization to application.

Jinling Tang1, Kunzhao Du2

  • 1Clinical Laboratory Center, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China.

Cell & Bioscience
|January 25, 2025
PubMed
Summary
This summary is machine-generated.

Mitochondrial DNA base editing systems, like DddA-derived cytosine base editors (DdCBEs), enable precise C-to-T and A-to-G conversions in mtDNA. These bioengineering tools show promise for applications in animal models and human embryos.

Keywords:
DdCBEsGenetic engineeringMitochondrial DNATALENsmtDNA base editing

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Area of Science:

  • Bioengineering
  • Molecular Biology
  • Genetics

Background:

  • Mitochondrial DNA (mtDNA) base editing systems are emerging bioengineering tools.
  • DddA-derived cytosine base editors (DdCBEs) and mtDNA adenine base editors facilitate targeted C-to-T and A-to-G conversions, respectively.

Purpose of the Study:

  • To review the principles and evolution of mtDNA base-editing systems.
  • To elaborate on different platforms, including deaminase replacement and DddAtox variant engineering.
  • To discuss applications and future challenges of mtDNA base editors.

Main Methods:

  • Review of existing literature on mtDNA base editing systems.
  • Analysis of DdCBEs and mtDNA adenine base editors.
  • Examination of deaminase replacement, DddAtox variants, and structural optimization.

Main Results:

  • Detailed summary of mtDNA base-editing principles and platforms.
  • Elaboration on engineering strategies for DddAtox variants and structure optimization.
  • Highlighting of editing outcomes and applications in preclinical models.

Conclusions:

  • Mitochondrial DNA base editing systems have evolved significantly, offering precise genetic modifications.
  • Applications in animal models and human embryos demonstrate therapeutic potential.
  • Future development requires addressing challenges in efficiency, specificity, and delivery.