Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis.

Molecular cell - 1 Jul 1999

Chang X B, Cui L, Hou Y X, Jensen T J, Aleksandrov A A, Mengos A, Riordan J R

Abstract excerpt

Many cystic fibrosis transmembrane conductance regulator (CFTR) mutants are recognized as aberrant by the quality control apparatus at the endoplasmic reticulum (ER) and are targeted for degradation. The mechanism whereby nascent chains are distinguished as either competent or incompetent for ER export has not been elucidated. Here we show that export-incompetent chains display multiple arginine-framed tripeptide...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Arginine
Share to Chloride Channels

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Chlorides

Share to Cystic Fibrosis Transmembrane Conductance Regulator

Share to Endoplasmic Reticulum

Share to Fluorescent Antibody Technique

Share to Lysine

Share to Mutagenesis, Site-Directed

Source

Identifiers

PMID: 10445036
DOI: 10.1016/s1097-2765(00)80196-3

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis.PMID 10445036Read ↗

F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive.Semantic proximity Read ↗

Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic ReticulumSemantic proximity Read ↗

Identification of molecular determinants that modulate trafficking of DeltaF508 CFTR, the mutant ABC transporter associated with cystic fibrosis.Semantic proximity Read ↗

Proteomics uncovering possible key players in F508del-CFTR processing and trafficking.Semantic proximity Read ↗

COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code.Semantic proximity Read ↗

Ribosomal frameshifting selectively modulates the assembly, function, and pharmacological rescue of a misfolded CFTR variant.Semantic proximity Read ↗

Interplay between ER exit code and domain conformation in CFTR misprocessing and rescue.Semantic proximity Read ↗

Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.Semantic proximity Read ↗

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

Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites.Semantic proximity Read ↗

CFTR processing, trafficking and interactions.Semantic proximity Read ↗

Inhibiting endoplasmic reticulum (ER)-associated degradation of misfolded Yor1p does not permit ER export despite the presence of a diacidic sorting signal.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.