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

Amyloid Fibrils03:03

Amyloid Fibrils

13.0K
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,...
13.0K
Amyloid Fibrils03:03

Amyloid Fibrils

6.9K
6.9K
Allosteric Proteins-ATCase01:19

Allosteric Proteins-ATCase

6.9K
Binding sites linkages can regulate a protein's function.  For example, enzyme activity is often regulated through a feedback mechanism where the end product of the biochemical process serves as an inhibitor.
Aspartate transcarbamoylase (ATCase) is a cytosolic enzyme that catalyzes the condensation of L-aspartate and carbamoyl phosphate to  N-carbamoyl-L-aspartate. This reaction is the first step in pyrimidine biosynthesis. UTP and CTP, the end products of the pyrimidine synthesis...
6.9K
Protein Complexes with Interchangeable Parts01:57

Protein Complexes with Interchangeable Parts

3.1K
Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order...
3.1K
Mutations01:39

Mutations

96.6K
Overview
96.6K
Mutations01:35

Mutations

45.5K
Mutations are changes in the sequence of DNA. These changes can occur spontaneously or they can be induced by exposure to environmental factors. Mutations can be characterized in a number of different ways: whether and how they alter the amino acid sequence of the protein, whether they occur over a small or large area of DNA, and whether they occur in somatic cells or germline cells.
Chromosomal Alterations Are Large-Scale Mutations
While point mutations are changes in a single nucleotide in...
45.5K

You might also read

Related Articles

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

Sort by
Same author

Oral hygiene management in critically ill patients: prevention of ventilator-associated pneumonia.

Frontiers in dental medicine·2026
Same author

Subcutaneous anesthetic technique to the auriculotemporal nerve for minimally invasive procedures in the temporomandibular joint.

International journal of oral and maxillofacial surgery·2026
Same author

Fatty acid profile and biological activity of supercritical fluid extract of <i>Rosa canina L.</i> seeds.

Natural product research·2025
Same author

Identification of prognostic liquid biopsy biomarkers in patients with cutaneous squamous cell carcinoma treated with cemiplimab.

Journal of translational medicine·2025
Same author

Cytoplasmic accumulation of a splice variant of hnRNPA2/B1 contributes to FUS-associated toxicity in a mouse model of ALS.

Cell death & disease·2025
Same author

The Interaction of Polygenic Susceptibility to Stress and Childhood Adversity Dimensions Predicts Longitudinal Trajectories of Stress-Sensitivity.

Stress and health : journal of the International Society for the Investigation of Stress·2024

Related Experiment Video

Updated: Mar 31, 2026

Sequential Extraction of Soluble and Insoluble Alpha-Synuclein from Parkinsonian Brains
09:27

Sequential Extraction of Soluble and Insoluble Alpha-Synuclein from Parkinsonian Brains

Published on: January 5, 2016

18.2K

Pur-alpha functionally interacts with FUS carrying ALS-associated mutations.

M Di Salvio1,2, V Piccinni1,2, V Gerbino3

  • 1IBPM, Istituto di Biologia e Patologia Molecolari, CNR, Rome, Italy.

Cell Death & Disease
|October 23, 2015
PubMed
Summary

Pur-alpha protein interacts with mutated FUS, exacerbating neurodegeneration in amyotrophic lateral sclerosis (ALS). Reducing Pur-alpha improves motor function in flies, suggesting its role in ALS pathogenesis via protein synthesis control.

More Related Videos

Identification and Classification of Position-specific GABAA Receptor Subunit Missense Variants for Their Role In Hippocampal Pyramidal Neurons
08:04

Identification and Classification of Position-specific GABAA Receptor Subunit Missense Variants for Their Role In Hippocampal Pyramidal Neurons

Published on: June 6, 2025

1.7K
Saccharomyces cerevisiae Models of Alzheimer's Disease to Screen Genes, Mutations, and Chemicals Affecting Amyloid Beta Production by &#947;-Secretase
11:57

Saccharomyces cerevisiae Models of Alzheimer's Disease to Screen Genes, Mutations, and Chemicals Affecting Amyloid Beta Production by γ-Secretase

Published on: June 24, 2025

694

Related Experiment Videos

Last Updated: Mar 31, 2026

Sequential Extraction of Soluble and Insoluble Alpha-Synuclein from Parkinsonian Brains
09:27

Sequential Extraction of Soluble and Insoluble Alpha-Synuclein from Parkinsonian Brains

Published on: January 5, 2016

18.2K
Identification and Classification of Position-specific GABAA Receptor Subunit Missense Variants for Their Role In Hippocampal Pyramidal Neurons
08:04

Identification and Classification of Position-specific GABAA Receptor Subunit Missense Variants for Their Role In Hippocampal Pyramidal Neurons

Published on: June 6, 2025

1.7K
Saccharomyces cerevisiae Models of Alzheimer's Disease to Screen Genes, Mutations, and Chemicals Affecting Amyloid Beta Production by &#947;-Secretase
11:57

Saccharomyces cerevisiae Models of Alzheimer's Disease to Screen Genes, Mutations, and Chemicals Affecting Amyloid Beta Production by γ-Secretase

Published on: June 24, 2025

694

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by motor neuron loss.
  • Mutations in the Fused in Sarcoma (FUS) protein are linked to ALS, causing it to aggregate in stress granules.

Purpose of the Study:

  • To investigate the role of Pur-alpha protein in the pathogenesis of FUS-associated ALS.
  • To explore the link between Pur-alpha, FUS aggregation, and protein synthesis dysregulation in ALS.

Main Methods:

  • Studied the interaction between Pur-alpha and mutated FUS in patient-derived motoneuronal cells.
  • Analyzed the effect of Pur-alpha and FUS on protein synthesis initiation factor eIF2alpha phosphorylation.
  • Utilized Drosophila models to assess the in vivo impact of Pur-alpha on FUS-induced neurodegeneration and motor function.

Main Results:

  • Pur-alpha physically interacts with mutated FUS in an RNA-dependent manner and colocalizes in stress granules.
  • Both Pur-alpha and mutated FUS upregulate eIF2alpha phosphorylation, inhibiting global protein synthesis.
  • Overexpression of Pur-alpha worsens FUS-induced neurodegeneration in Drosophila, while its downregulation improves motor function.

Conclusions:

  • Pur-alpha plays a significant role in the pathogenesis of FUS-associated ALS, likely through the regulation of mRNA translation.
  • Altered protein synthesis is implicated in ALS, with Pur-alpha emerging as a key factor in motoneuronal dysfunction.