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Related Experiment Videos

New developments in RAN translation: insights from multiple diseases.

John Douglas Cleary1, Laura Pw Ranum2

  • 1Center for NeuroGenetics, University of Florida, Gainesville, FL, USA; Department of Molecular Genetics & Microbiology, College of Medicine, University of Florida, Gainesville, FL, USA; Genetics Institute, University of Florida, Gainesville, FL, USA.

Current Opinion in Genetics & Development
|April 3, 2017
PubMed
Summary

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Repeat-associated non-ATG (RAN) translation produces proteins from repeat expansion mutations without standard initiation, contributing to neurodegenerative diseases like ALS/FTD.

Area of Science:

  • Molecular Biology
  • Neuroscience
  • Genetics

Background:

  • Repeat-associated non-ATG (RAN) translation is a non-canonical translation process.
  • RAN translation is implicated in neurodegenerative diseases, including amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD).
  • It involves the production of proteins from repeat expansion mutations in all three reading frames without an ATG initiation codon.

Purpose of the Study:

  • To review recent developments in RAN translation.
  • To explore the mechanistic complexity of RAN translation.
  • To discuss the downstream consequences of RAN translation in disease and gene expression.

Main Methods:

  • Review of existing literature on RAN translation and associated diseases.
  • Analysis of studies investigating different types of repeat expansion mutations (e.g., CAG, CTG, CCG, GGGGCC, GGCCCC).

Related Experiment Videos

  • Examination of the interplay between bidirectional transcription and RAN translation.

    • Main Results:

    • RAN translation generates multiple mutant proteins from repeat expansions.
    • Up to six distinct mutant expansion proteins can accumulate due to bidirectional transcription and RAN translation.
    • This process contributes to the pathogenesis of various diseases.

    Conclusions:

    • RAN translation represents a complex molecular mechanism with significant implications for neurodegenerative diseases.
    • Understanding RAN translation is crucial for advancing our knowledge of gene expression and disease.
    • Further research is needed to fully elucidate the downstream effects and therapeutic potential.