Related Experiment Video

Updated: Jun 27, 2025

Visualization of DNA Repair Proteins Interaction by Immunofluorescence
07:55

Visualization of DNA Repair Proteins Interaction by Immunofluorescence

Published on: June 26, 2020

10.2K

[Research Progress in the Roles of MRE11-RAD50-NBS1 Complex and Human Diseases]

Xiao-Hui Xu1, Yi-Dan Liu1

  • 1Women's Hospital,School of Medicine,Zhejiang University,Hangzhou 310006,China.

Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
|April 30, 2024
PubMed

Insights

The MRE11-RAD50-NBS1 (MRN) complex is crucial for accurate DNA double-strand break repair via homologous recombination. Dysfunctions in this complex lead to severe human genetic disorders, studied using mouse models.

Area of Science:

  • Molecular Biology
  • Genetics
  • Cell Biology

Background:

  • DNA double-strand breaks (DSBs) are highly deleterious DNA lesions.
  • Homologous recombination (HR) is a high-fidelity DNA repair pathway.
  • The MRE11-RAD50-NBS1 (MRN) complex is a key conserved mediator of HR.

Purpose of the Study:

  • To review the composition, structure, and HR roles of the MRN complex.
  • To discuss human diseases linked to MRN complex dysfunction.
  • To summarize mouse models for studying MRN-related disorders.

Main Methods:

  • Literature review of MRN complex function in DNA repair.
  • Analysis of genetic disorders associated with MRN complex deficiencies.
  • Compilation of data from established mouse models.

Main Results:

  • The MRN complex is essential for HR and genomic stability in higher animals.
  • MRN complex dysfunction underlies ataxia-telangiectasia-like disorder, Nijmegen breakage syndrome, and related disorders.
  • Mouse models recapitulate key clinical features of these human diseases.

Conclusions:

  • The MRN complex is a critical target for understanding DNA repair and associated human diseases.
  • Further research using MRN complex models will elucidate disease mechanisms and therapeutic strategies.
Keywords:
DNA double-strand breakDNA repairMRE11-RAD50-NBS1 complexhomologous recombination

More Related Videos

Real-time Observation of the DNA Strand Exchange Reaction Mediated by Rad5106:24

Real-time Observation of the DNA Strand Exchange Reaction Mediated by Rad51

Published on: February 13, 2019

8.0K
Assessment of Global DNA Double-Strand End Resection using BrdU-DNA Labeling coupled with Cell Cycle Discrimination Imaging06:44

Assessment of Global DNA Double-Strand End Resection using BrdU-DNA Labeling coupled with Cell Cycle Discrimination Imaging

Published on: April 28, 2021

4.0K

Related Concept Videos

Mismatch Repair01:20

Mismatch Repair

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.
The Mutator Protein Family Plays a Key Role in DNA Mismatch Repair
The human genome has more than 3 billion base pairs of DNA per cell. Prior to cell division, that vast amount of genetic...
4.8K
Nucleotide Excision Repair01:38

Nucleotide Excision Repair

DNA Distortion and Damage
Cells are regularly exposed to mutagens—factors in the environment that can damage DNA and generate mutations. UV radiation is one of the most common mutagens and is estimated to introduce a significant number of changes in DNA. These include bends or kinks in the structure, which can block DNA replication or transcription. If these errors are not fixed, the damage can cause mutations, which in turn can result in cancer or disease depending on which sequences are...
3.5K
Homologous Recombination02:31

Homologous Recombination

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...
50.5K
DNA Helicases00:55

DNA Helicases

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...
21.3K
Regulation of the Unfolded Protein Response01:31

Regulation of the Unfolded Protein Response

Inositol-requiring kinase one or IRE1 is the most conserved eukaryotic unfolded protein response (UPR) receptor. It is a type I transmembrane protein kinase receptor with a distinctive site-specific RNase activity. As the binding mechanics of the misfolded proteins with the N-terminal domain of IRE-1 are unclear, three binding models — direct, indirect, and allosteric -- are proposed for receptor activation. Nevertheless, it is known that once a misfolded protein associates with IRE1, it...
2.4K
Long-patch Base Excision Repair01:02

Long-patch Base Excision Repair

Since the discovery of the two BER pathways, there has been a debate about how a cell chooses one pathway over the other and the factors determining this selection. Numerous in vitro experiments have pointed out multiple determinants for the sub-pathway selection. These are:
7.0K