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Article

Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.

American journal of physiology. Cell physiology - 1 Feb 2000

Rubenstein R C, Zeitlin P L

Abstract excerpt

The most common mutation of the cystic fibrosis transmembrane conductance regulator (CFTR), DeltaF508, is a trafficking mutant that has prolonged associations with molecular chaperones and is rapidly degraded, at least in part by the ubiquitin-proteasome system. Sodium 4-phenylbutyrate (4PBA) improves DeltaF508-CFTR trafficking and function in vitro in cystic fibrosis epithelial cells and in vivo. To further...

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Source

Identifiers

PMID: 10666020
DOI: 10.1152/ajpcell.2000.278.2.C259

Genes

CFTR

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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.

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Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.PMID 10666020Read ↗

Induction of HSP70 promotes DeltaF508 CFTR trafficking.Semantic proximity Read ↗

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Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.Semantic proximity Read ↗

The endoplasmic reticulum–associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3–dependent degradation of nascent CFTRΔF508Semantic proximity Read ↗

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Targeting ubiquitination machinery in cystic fibrosis: Where do we stand?Semantic proximity Read ↗

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