Related Experiment Video
Updated: Jul 31, 2025

In Vitro Analysis of E3 Ubiquitin Ligase Function
Published on: May 14, 2021
The multiple ubiquitination mechanisms in CFTR peripheral quality control
Shogo Taniguchi1, Ryosuke Fukuda1, Tsukasa Okiyoneda1
1Department of Biomedical Sciences, School of Biological and Environmental Sciences, Kwansei Gakuin University, Hyogo 669-1330, Japan.
Targeting peripheral protein quality control (PeriQC) degradation of cystic fibrosis transmembrane conductance regulator (CFTR) offers a new therapeutic strategy. Counteracting CFTR ubiquitination in PeriQC can enhance treatment efficacy for cystic fibrosis.
Area of Science:
- Biochemistry
- Cell Biology
- Genetics
Background:
- Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated anion channel crucial for epithelial cell function.
- Mutations in CFTR cause cystic fibrosis (CF), a common genetic disorder, often leading to protein misfolding and degradation.
- Current therapies face limitations as even PM-localized CFTR is degraded by peripheral protein quality control (PeriQC).
Conclusions:
- Targeting CFTR ubiquitination in PeriQC presents a promising therapeutic avenue for cystic fibrosis.
- Developing strategies to counteract PeriQC-mediated degradation can enhance the efficacy of existing and future CF treatments.
- Further research into CFTR PeriQC mechanisms may unlock novel treatment modalities.
Related Concept Videos
Regulated Protein Degradation
Protein degradation plays two important roles in the cells. It helps to protect cells from misfolded or damaged proteins before they lead to a...
Protein Folding Quality Check in the RER
Export of Misfolded Proteins out of the ER
Covalently Linked Protein Regulators
These groups modify specific amino acids in a protein....
The Proteasome
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...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...

