Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.

doi:10.1371/journal.pone.0166948

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Article

Endoplasmic Reticulum-Targeted Subunit Toxins Provide a New Approach to Rescue Misfolded Mutant Proteins and Revert Cell Models of Genetic Diseases.

PloS one - 1 Jan 2016

Adnan Humaira, Zhang Zhenbo, Park Hyun-Joo, Tailor Chetankumar, Che Clare, Kamani Mustafa, Spitalny George, Binnington Beth, Lingwood Clifford

Abstract excerpt

Many germ line diseases stem from a relatively minor disturbance in mutant protein endoplasmic reticulum (ER) 3D assembly. Chaperones are recruited which, on failure to correct folding, sort the mutant for retrotranslocation and cytosolic proteasomal degradation (ER-associated degradation-ERAD), to initiate/exacerbate deficiency-disease symptoms. Several bacterial (and plant) subunit toxins, retrograde transport...

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Topics

Blotting, Western
Cholera Toxin
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Endoplasmic Reticulum

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Endoplasmic Reticulum-Associated Degradation

HEK293 Cells

Humans

Microscopy, Fluorescence

Models, Biological

Mutation

Protein Folding

Protein Transport

Proteostasis Deficiencies

Shiga Toxins

Toxins, Biological

Source

Open in PubMed Open full text

Identifiers

PMID: 27935997
DOI: 10.1371/journal.pone.0166948
PMCID: PMC5147855

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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