Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Translation01:31

Translation

21.6K
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 Life
Proteins are...
21.6K
Translation01:31

Translation

159.3K
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...
159.3K
Amyloid Fibrils03:03

Amyloid Fibrils

12.7K
Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
12.7K
Leaky Scanning02:28

Leaky Scanning

5.8K
During most eukaryotic translation processes, the small 40S ribosome subunit scans an mRNA from its 5' end until it encounters the first start AUG codon. The large 60S ribosomal subunit then joins the smaller one to initiate protein synthesis. The location of the translation initiation is largely determined by the nucleotides near the start codon as there may be multiple translation initiation sites present on the mRNA.  Marilyn Kozak discovered that the sequence RCCAUGG (where R...
5.8K
RNA Editing02:23

RNA Editing

10.1K
RNA editing is a post-transcriptional modification where a precursor mRNA (pre-mRNA) nucleotide sequence is changed by base insertion, deletion, or modification. The extent of RNA editing varies from a few hundred bases, in mitochondrial DNA of trypanosomes, to a just single base, in nuclear genes of mammals. Even a single base change in the pre-mRNA can convert a codon for one amino acid into the codon for another amino acid or a stop codon. This type of re-coding can significantly affect the...
10.1K
Alternative RNA Splicing02:18

Alternative RNA Splicing

25.9K
Alternative RNA splicing is the regulated splicing of exons and introns to produce different mature mRNAs from a single pre-mRNA. Unlike in constitutive splicing where a single gene produces a single type of mRNA, alternative splicing allows an organism to produce multiple proteins from a single gene and plays an important role in protein diversity.
There are five types of alternative RNA splicing that vary in the ways the pre-mRNA segments are removed or retained in the mature mRNA. The first...
25.9K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

ANYI: The ANnotated Yeast Interactome.

bioRxiv : the preprint server for biology·2026
Same author

Detecting misfolded non-covalent lasso entanglements in protein structures, simulation trajectories, and mass spectrometry data.

bioRxiv : the preprint server for biology·2026
Same author

Native entanglement misfolding contributes to age-associated structural change<i>s</i> across the <i>Saccharomyces cerevisiae</i> proteome.

bioRxiv : the preprint server for biology·2026
Same author

Protein entanglement misfolding determines divergent fates: proteasomal degradation or persistence in near-native misfolded states.

bioRxiv : the preprint server for biology·2026
Same author

Natively entangled proteins are linked to human disease and pathogenic mutations likely due to a greater misfolding propensity.

bioRxiv : the preprint server for biology·2026
Same author

iNOS modulates inflammatory responses in an NO-independent manner through direct interaction with IRG1 in mitochondria.

Nature metabolism·2026

Related Experiment Video

Updated: Mar 17, 2026

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
07:08

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

10.1K

Altered Co-Translational Processing Plays a Role in Huntington's Pathogenesis-A Hypothesis.

Daniel A Nissley1, Edward P O'Brien1

  • 1O'Brien Lab, Department of Chemistry, The Pennsylvania State University University Park, PA, USA.

Frontiers in Molecular Neuroscience
|July 27, 2016
PubMed
Summary

Huntington's disease (HD) is linked to altered protein production kinetics. Our hypothesis suggests that changes in translation speed due to CAG repeat expansion disrupt huntingtin protein localization and cell function, offering new therapeutic targets.

Keywords:
Huntington's diseasebiophysicskineticsneurodegenerative diseaseprotein aggregationprotein biogenesistranslationtranslation regulation

More Related Videos

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System
10:52

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System

Published on: December 10, 2021

3.1K
Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy
11:22

Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy

Published on: June 27, 2018

8.5K

Related Experiment Videos

Last Updated: Mar 17, 2026

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
07:08

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

10.1K
Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System
10:52

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System

Published on: December 10, 2021

3.1K
Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy
11:22

Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy

Published on: June 27, 2018

8.5K

Area of Science:

  • Neurodegenerative diseases
  • Molecular biology
  • Genetics

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder.
  • It is caused by CAG repeat expansion in the HTT gene, leading to mutant huntingtin protein aggregation and neuronal death.
  • The precise molecular mechanisms linking genotype to phenotype in HD remain unclear, hindering therapeutic development.

Purpose of the Study:

  • To propose a novel hypothesis for Huntington's disease pathogenesis.
  • To investigate the role of co-translational processes and translation-elongation kinetics in HD.
  • To explore how CAG repeat expansion affects huntingtin protein biogenesis and cellular function.

Main Methods:

  • The study proposes a hypothesis based on existing research in protein biogenesis.
  • It analyzes the impact of CAG repeat expansion on translational pause sites and kinetics.
  • The hypothesis is evaluated for consistency with known HD experimental observations.

Main Results:

  • The hypothesis posits that altered translation-elongation kinetics due to CAG repeat expansion perturb co-translational processes.
  • This perturbation shifts a proline-induced translational pause site away from the huntingtin protein's localization sequence.
  • The hypothesis explains the correlation between CAG repeat length and the age of HD symptom onset.

Conclusions:

  • The proposed hypothesis offers a new perspective on HD molecular pathology.
  • It highlights the critical role of co-translational processes in huntingtin protein regulation.
  • The study suggests specific experiments to validate the proposed mechanism and explore therapeutic strategies.