Related Experiment Video
Updated: Jul 5, 2026
06:09
Studying the Protein Quality Control System of D. discoideum Using Temperature-controlled Live Cell Imaging
Published on: December 2, 2016
Quality control in the endoplasmic reticulum protein factory
Roberto Sitia1, Ineke Braakman
1Università Vita-Salute San Raffaele Scientific Institute, 20132 Milano, Italy. r.sitia@hsr.it
Nature
|December 20, 2003
Summary
The endoplasmic reticulum (ER) quality control ensures proper protein folding. Its systems and chaperones are key targets for treating protein-folding diseases like amyloidosis.
Area of Science:
- Cell Biology
- Molecular Biology
- Biochemistry
Background:
- The endoplasmic reticulum (ER) functions as a cellular factory for synthesizing secretory proteins.
- It employs rigorous quality control mechanisms to ensure correct protein folding before secretion.
- This process is crucial for cellular function and preventing disease.
Purpose of the Study:
- To highlight the endoplasmic reticulum's role in protein quality control.
- To discuss the signaling pathways that regulate ER folding assistants.
- To identify ER chaperones and signaling molecules as potential therapeutic targets.
Main Methods:
- Review of current literature on ER protein folding and quality control.
- Analysis of signaling pathways involved in ER homeostasis.
- Identification of drug targets in protein-conformational diseases.
Main Results:
- The ER utilizes integrated signaling pathways to adapt to changing needs.
- These pathways modulate the levels of folding assistants, such as ER chaperones.
- Dysfunctional protein folding in the ER is implicated in various diseases.
Conclusions:
- ER chaperones and signaling molecules are critical for protein homeostasis.
- Targeting these components offers a promising therapeutic strategy for amyloidoses and other protein-conformational diseases.
- Understanding ER quality control is vital for developing treatments for protein misfolding disorders.
More Related Videos
Related Concept Videos
01:29Protein Folding Quality Check in the RER
ER is the primary site for the maturation and folding of soluble and transmembrane secretory proteins. The calnexin cycle is a specific chaperone system that folds and assesses the confirmation of N-glycosylated proteins before they can exit the ER lumen. The primary players of this quality check pipeline are the lectins, ER-resident chaperones, and a glucosyl transferase enzyme. In case the calnexin system in the lumen fails to salvage a misfolded protein, it is transported to the cytoplasm...
01:05Quality Control
Quality control is one of the three cyclical quality assurance activities that help keep a system under statistical control. Typical quality control activities include creating quality control charts, conducting proficiency testing, and documenting and archiving results.
Quality control helps track data, visualize trends, and identify variations, making it easier to detect deviations that may affect the accuracy of an analysis. One way to do this is by generating a quality control chart, which...
Quality control helps track data, visualize trends, and identify variations, making it easier to detect deviations that may affect the accuracy of an analysis. One way to do this is by generating a quality control chart, which...
01:19Quality Assurance
Quality assurance is the overarching term used to describe the activities employed to ensure the proper performance of a system. These activities can be classified into three categories: quality control, quality assessment, and internal corrective measures. Typically, these activities work cyclically: quality control is performed before and during the analysis, while quality assessment occurs during and after the investigation. Internal corrective measures are implemented based on the findings...
01:26Bacterial Protein Maturation
Bacterial protein maturation is a tightly regulated process that ensures newly synthesized polypeptides achieve correct functional conformations. This maturation involves a series of modifications, folding events, and quality control steps, often assisted by specialized chaperone proteins.N-Terminal ModificationsThe maturation of bacterial polypeptides begins cotranslationally as the polypeptide exits the ribosome. The first amino acid, N-formylmethionine (fMet), is typically modified at the...
01:26Good Manufacturing Practices
Good Manufacturing Practices (GMP) constitute a foundational set of guidelines that ensure the production of safe, consistent, and high-quality products, particularly in industries such as pharmaceuticals, biotechnology, and food processing. These protocols encompass all aspects of production, from the sourcing of raw materials to the final distribution of the finished product.A core pillar of GMP is stringent hygiene and sanitation across all production environments. This includes routine...
01:28Bioreactor Controls-I
Maintaining optimal conditions within fermenters is essential for maximizing microbial productivity and ensuring process efficiency. This lesson focuses on key parameters—temperature, foam, pH, carbon dioxide, oxygen, and pressure—and their precise measurement and control strategies in fermentation systems.Temperature ControlTemperature regulation is critical due to the exothermic nature of many fermentation processes. In small laboratory fermenters, temperature is commonly monitored using...