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Article

Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking.

Journal of cell science - 1 Feb 2007

Gentzsch Martina, Choudhury Amit, Chang Xiu-bao, Pagano Richard E, Riordan John R

Abstract excerpt

Most patients with cystic fibrosis (CF) have a single codon deletion (DeltaF508) in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) that impairs assembly of the multidomain glycoprotein. The mutant protein escapes endoplasmic reticulum (ER) quality control at low temperature, but is rapidly cleared from the distal secretory pathway and degraded in lysosomes. CF cells accumulate...

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Identifiers

PMID: 17213331
DOI: 10.1242/jcs.03350

Genes

CFTR

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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.

Related research

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Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid trafficking.PMID 17213331Read ↗

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Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein.Semantic proximity Read ↗

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