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Related Concept Videos

Translation01:31

Translation

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Lesson: Translation
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of...
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Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
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Termination of Translation

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The large ribosomal subunit has several important structures essential to translation. These include the peptidyl transferase center (PTC) - which is the site where the peptide bond is formed - and a large, internal, water-filled tube through which the nascent polypeptide moves. This latter structure is called the Peptide Exit Tunnel, and it begins at the PTC and spans the body of the large ribosomal subunit. During translation, as the nascent polypeptide chain is synthesized, it passes through...
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The period of muscle contraction primarily influences the duration of stimulation at the neuromuscular junction (NMJ), the presence of free calcium ions in the sarcoplasm, and the availability of energy or ATP to support contractions.
When an action potential reaches the axon terminal, it depolarizes the membrane and opens voltage-gated sodium channels. Sodium ions enter the cell, further depolarizing the presynaptic membrane. This depolarization causes voltage-gated calcium channels to open....
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Directionality of Nuclear Transport01:42

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Ras-related nuclear protein or Ran is a small G protein that cycles between its GTP and GDP bound states. Ran specific regulators, a Ran GTPase Activating Protein or RanGAP present in the cytosol and a Ran guanine nucleotide exchange factor or RanGEF present inside the nucleus regulate GTP/GDP exchange. A high concentration of GTP inside the cells, in addition to this asymmetric distribution of  Ran-specific regulators, leads to a higher RanGTP concentration inside the nucleus. This...
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Relax, Don't RAN Translate It.

Katherine M Wilson1, Bhavana Muralidharan2, Adrian M Isaacs1

  • 1UK Dementia Research Institute at UCL, Gower Street, London WC1E 6BT, UK; Department of Neurodegenerative Disease, Queen Square UCL Institute of Neurology, London WC1N 3BG, UK.

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Summary
This summary is machine-generated.

The C9orf72 (GGGGCC)n repeat expansion causes frontotemporal dementia and ALS via repeat-associated non-AUG (RAN) translation. The helicase DDX3X unwinds RNA and was found to suppress this toxic RAN translation.

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Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Background:

  • The C9orf72 (GGGGCC)n repeat expansion is the leading genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS).
  • This repeat expansion is translated into toxic proteins via a non-canonical process termed repeat-associated non-AUG (RAN) translation.
  • Understanding the molecular mechanisms regulating RAN translation is crucial for developing therapeutic strategies for C9orf72-associated neurodegenerative diseases.

Purpose of the Study:

  • To investigate the role of RNA helicases in modulating C9orf72 repeat-associated non-AUG (RAN) translation.
  • To identify specific RNA helicases that can suppress the production of dipeptide repeat proteins (DPRs) from the C9orf72 repeat expansion.
  • To determine the functional impact of identified RNA helicases on cellular toxicity associated with C9orf72 repeat expansions.

Main Methods:

  • Utilized cell-based models expressing the C9orf72 (GGGGCC)n repeat expansion.
  • Employed techniques such as Western blotting and immunofluorescence to detect and quantify RAN translation products (DPRs).
  • Assessed the impact of RNA helicase knockdown or overexpression on DPR levels and cellular viability.

Main Results:

  • The RNA helicase DDX3X was identified as a key regulator of C9orf72 RAN translation.
  • Overexpression of DDX3X significantly suppressed the production of various DPRs, including poly(GP) and poly(GA).
  • Knockdown of DDX3X led to increased DPR levels and enhanced cellular toxicity, indicating a protective role for DDX3X.

Conclusions:

  • DDX3X acts as a suppressor of C9orf72 repeat-associated non-AUG (RAN) translation.
  • Targeting DDX3X or its regulatory pathways may offer a therapeutic avenue for C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis.
  • These findings elucidate a novel mechanism controlling the toxicity of repeat expansion disorders.