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Related Concept Videos

DNA Helicases00:55

DNA Helicases

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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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DNA Topoisomerases02:02

DNA Topoisomerases

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Topoisomerases are enzymes that relax overwound DNA molecules during various cell processes, including DNA replication and transcription. These enzymes regulate positive and negative DNA supercoiling without changing the nucleotide sequence. DNA overwinding in a clockwise direction results in positively supercoiled DNA, whereas underwinding in a counterclockwise direction produces negatively supercoiled DNA.
Types and Mechanism of action
Topoisomerases are divided into two main types. ...
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Single-Strand DNA Binding Proteins01:03

Single-Strand DNA Binding Proteins

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For successful DNA replication, the unwinding of double-stranded DNA must be accompanied by stabilization and protection of the separated single strands of the DNA. This crucial task is performed by single-strand DNA-binding (SSB) proteins. They bind to the DNA in a sequence-independent manner, which means that the nitrogenous bases of the DNA need not be present in a specific order for binding of SSB proteins to it. The binding of SSB proteins straightens single-stranded DNA (ssDNA) and makes...
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Homologous Recombination02:31

Homologous Recombination

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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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Restarting Stalled Replication Forks02:37

Restarting Stalled Replication Forks

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DNA replication is initiated at sites containing predefined DNA sequences known as origins of replication. DNA is unwound at these sites by the minichromosome maintenance (MCM) helicase and other factors such as Cdc45 and the associated GINS complex.The unwound single strands are protected by replication protein A (RPA) until DNA polymerase starts synthesizing DNA at the 5’ end of the strand in the same direction as the replication fork. To prevent the replication fork from falling apart,...
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DNA Damage can Stall the Cell Cycle02:36

DNA Damage can Stall the Cell Cycle

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In response to DNA damage, cells can pause the cell cycle to assess and repair the breaks. However, the cell must check the DNA at certain critical stages during the cell cycle. If the cell cycle pauses before DNA replication, the cells will contain twice the amount of DNA. On the other hand, if cells arrest after DNA replication but before mitosis, they will contain four times the normal amount of DNA. With a host of specialized proteins at their disposal,cells must use the right protein at...
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Tools to Study the Role of Architectural Protein HMGB1 in the Processing of Helix Distorting, Site-specific DNA Interstrand Crosslinks
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RECQ DNA helicases and osteosarcoma.

Linchao Lu1, Weidong Jin, Hao Liu

  • 1Section of Hematology/Oncology, Department of Pediatrics, Texas Children's Cancer Center, Baylor College of Medicine, 1102 Bates Avenue, Suite 1200, Houston, TX, 77030, USA, linchaol@bcm.edu.

Advances in Experimental Medicine and Biology
|June 14, 2014
PubMed
Summary

The RECQ helicase RECQL4 is crucial for genomic integrity and its dysfunction is linked to Rothmund-Thomson syndrome (RTS), a cancer predisposition disorder. Understanding RECQL4

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Area of Science:

  • Genetics and Molecular Biology
  • Cancer Biology
  • Genomic Stability

Background:

  • The RECQ family of DNA helicases maintains genomic integrity.
  • Mutations in human RECQ helicase genes (BLM, WRN, RECQL4) cause genetic disorders: Bloom syndrome, Werner syndrome, and Rothmund-Thomson syndrome (RTS).
  • RTS is a cancer predisposition syndrome with unique susceptibility to osteosarcoma, making it a model for studying osteosarcoma pathogenesis.

Purpose of the Study:

  • To review the cellular functions of RECQL4.
  • To explore the relationship between RECQL4 functions and tumorigenesis.
  • To summarize in vivo studies of RECQL4 using animal models.

Main Methods:

  • Literature review of cellular and molecular functions of RECQL4.
  • Analysis of the role of RECQL4 in genomic stability and DNA repair.
  • Examination of data from animal models investigating RECQL4's in vivo functions.

Main Results:

  • RECQL4 plays a role in maintaining genomic integrity.
  • Dysfunction of RECQL4 is associated with cancer predisposition, particularly osteosarcoma in RTS patients.
  • Ongoing research using animal models is elucidating RECQL4's in vivo functions.

Conclusions:

  • RECQL4, as a RECQ helicase, has a tumor suppressor role.
  • Understanding RECQL4 pathways may lead to novel cancer therapies.
  • Further research into RECQL4's cellular and in vivo functions is critical for advancing cancer treatment strategies.