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

The Proteasome01:13

The Proteasome

2.0K
Eukaryotic cells can degrade proteins through several pathways. One of the most important among these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. This involves participation of a series of enzymes including— E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...
2.0K
The Proteasome02:18

The Proteasome

10.5K
Eukaryotic cells can degrade proteins through several pathways. One of the most important amongst these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. A series of enzymes carry out the ubiquitination of the target proteins - E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...
10.5K
The Proteasome02:18

The Proteasome

5.0K
5.0K
Overview of Protein Metabolism01:21

Overview of Protein Metabolism

4.6K
Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
4.6K
Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

997
Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
997
Notch Signaling Pathway03:14

Notch Signaling Pathway

6.8K
The Notch signaling pathway is a major intracellular signaling pathway that is highly conserved over a broad spectrum of metazoan species. It stands unique from other intracellular signaling mechanisms in animals because notch protein itself acts as the receptor as well as the primary signaling molecule.
The Notch gene came into the limelight in 1914 after the discovery that its mutation in Drosophila melanogaster leads to a serrated (or "notched") wing margin phenotype. It was not...
6.8K

You might also read

Related Articles

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

Sort by
Same author

Behavioral Thermoceptive Responses and Morphologic Correlates in Mouse Models of CMT1A, HNPP, and Aging.

Journal of the peripheral nervous system : JPNS·2026
Same author

The Calcium Connection: Explaining Motor Neuron Vulnerability in ALS.

Cells·2026
Same author

Engineered GM1 Intersects Between Mitochondrial and Synaptic Pathways to Ameliorate ALS Pathology.

Advanced science (Weinheim, Baden-Wurttemberg, Germany)·2026
Same author

The Neuromuscular Junction: A Shared Vulnerability in Aging and Disease.

The Journal of neuroscience : the official journal of the Society for Neuroscience·2025
Same author

Innovations In Physical Medicine and Rehabilitation: Advances in the Diagnosis, Treatment, and Care of Amyotrophic Lateral Sclerosis.

Missouri medicine·2025
Same author

Microglia in ALS: Insights into Mechanisms and Therapeutic Potential.

Cells·2025

Related Experiment Video

Updated: Mar 23, 2026

Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans
09:18

Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans

Published on: September 7, 2021

3.4K

Proteostasis impairment in ALS.

Céline Ruegsegger1, Smita Saxena2

  • 1Institute of Cell Biology, University of Bern, Baltzerstrasse 4, CH-3012 Bern, Switzerland; Graduate School for Cellular and Biomedical Sciences, University of Bern, CH-3012 Bern, Switzerland.

Brain Research
|April 2, 2016
PubMed
Summary

The proteostasis network maintains cellular health by managing protein folding and clearance. Its failure is linked to aging disorders, including neurodegenerative diseases like Amyotrophic Lateral Sclerosis (ALS).

Keywords:
ALSAutophagyER stressMotoneuronNeurodegenerationProteasomeProteostasis

More Related Videos

Assays for the Degradation of Misfolded Proteins in Cells
10:56

Assays for the Degradation of Misfolded Proteins in Cells

Published on: August 28, 2016

12.7K
Using Caenorhabditis elegans as a Model System to Study Protein Homeostasis in a Multicellular Organism
12:38

Using Caenorhabditis elegans as a Model System to Study Protein Homeostasis in a Multicellular Organism

Published on: December 18, 2013

6.6K

Related Experiment Videos

Last Updated: Mar 23, 2026

Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans
09:18

Quantifying Tissue-Specific Proteostatic Decline in Caenorhabditis elegans

Published on: September 7, 2021

3.4K
Assays for the Degradation of Misfolded Proteins in Cells
10:56

Assays for the Degradation of Misfolded Proteins in Cells

Published on: August 28, 2016

12.7K
Using Caenorhabditis elegans as a Model System to Study Protein Homeostasis in a Multicellular Organism
12:38

Using Caenorhabditis elegans as a Model System to Study Protein Homeostasis in a Multicellular Organism

Published on: December 18, 2013

6.6K

Area of Science:

  • Cellular Biology
  • Molecular Biology
  • Neuroscience

Background:

  • Cellular proteostasis is crucial for cell function and survival.
  • Cells adapt to environmental stressors using molecular chaperones and clearance pathways.
  • The proteostasis network prevents protein misfolding, aggregation, and accumulation.

Purpose of the Study:

  • To review the proteostasis process.
  • To explore how proteostasis failure contributes to neurodegenerative disorders.
  • To focus specifically on Amyotrophic Lateral Sclerosis (ALS).

Main Methods:

  • Literature review of proteostasis mechanisms.
  • Analysis of the link between proteostasis impairment and disease.
  • Focus on cellular stress responses and their role in ALS.

Main Results:

  • Proteostasis network components include chaperones, clearance pathways, and signaling networks.
  • Imbalances in proteostasis are associated with aging and various diseases.
  • Proteostasis failure is a key factor in the pathogenesis of neurodegenerative diseases, particularly ALS.

Conclusions:

  • Maintaining proteostasis is vital for preventing cellular dysfunction and disease.
  • Understanding proteostasis failure offers insights into treating neurodegenerative conditions like ALS.
  • This review highlights the critical role of the proteostasis network in health and disease.