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DNA Helicases00:55

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DNA unwinding helicase enzymes are a type of motor protein. Motor proteins can translocate along filaments or polymers using energy generated from ATP hydrolysis. Helicases are involved in all the important cellular processes where DNA unwinding is required, such as DNA replication, repair, recombination, and transcription. They are present in all living organisms, but vary in their structure, function, and mechanism of action. For example, in prokaryotes, DnaB helicase binds and translocates...
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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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Mismatch Repair01:20

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Organisms are capable of detecting and fixing nucleotide mismatches that occur during DNA replication. This sophisticated process requires identifying the new strand and replacing the erroneous bases with correct nucleotides. Mismatch repair is coordinated by many proteins in both prokaryotes and eukaryotes.
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The basic reaction of homologous recombination (HR) involves two chromatids that contain DNA sequences sharing a significant stretch of identity. One of these sequences uses a strand from another as a template to synthesize DNA in an enzyme-catalyzed reaction. The final product is a novel amalgamation of the two substrates. To ensure an accurate recombination of sequences, HR is restricted to the S and G2 phases of the cell cycle. At these stages, the DNA has been replicated already and the...
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Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA
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Simultaneous Mapping and Quantitation of Ribonucleotides in Human Mitochondrial DNA

Published on: November 14, 2017

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Human mitochondrial nucleases.

Francesco Bruni1, Robert N Lightowlers1, Zofia M Chrzanowska-Lightowlers1

  • 1The Wellcome Trust Centre for Mitochondrial Research, The Medical School, Newcastle University, UK.

The FEBS Journal
|December 8, 2016
PubMed
Summary
This summary is machine-generated.

Mitochondria maintain essential functions through their DNA (mtDNA). Nucleases degrade mtDNA and mtRNA, and mutations in these enzymes cause mitochondrial diseases.

Keywords:
mtDNAcleavagedegradationhumanmitochondriamtRNAnucleasesprocessing

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Area of Science:

  • Cellular Biology
  • Molecular Biology
  • Genetics

Background:

  • Mitochondria are vital organelles with numerous cellular roles, including energy production and apoptosis.
  • They possess a unique extrachromosomal mitochondrial genome (mtDNA) and the machinery for its expression.
  • Maintaining mitochondrial gene expression requires balancing mtDNA and mtRNA synthesis with degradation.

Purpose of the Study:

  • To investigate the role of mitochondrial nucleases in maintaining mitochondrial gene expression.
  • To identify nucleases involved in mtDNA and mtRNA degradation.
  • To explore the link between mutations in nuclease genes and pathogenic mitochondrial diseases.

Main Methods:

  • Characterization of mitochondrial nucleases.
  • Analysis of mtDNA and mtRNA degradation pathways.
  • Genetic studies linking nuclease mutations to mitochondrial dysfunction.

Main Results:

  • Several mitochondrial nucleases have been identified, but their roles in DNA and RNA degradation are not fully understood.
  • The balance of mtDNA and mtRNA levels is crucial for proper mitochondrial function.
  • Mutations in genes encoding specific nucleases are associated with the development of mitochondrial diseases.

Conclusions:

  • Mitochondrial nucleases play a critical role in regulating mitochondrial gene expression by degrading mtDNA and mtRNA.
  • Further characterization of these nucleases is essential for understanding mitochondrial health.
  • Identifying nuclease defects offers potential targets for diagnosing and treating mitochondrial diseases.