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Article

BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-like-domain-dependent manner.

Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology - 1 Jan 2012

Mendes Filipa, Farinha Carlos M, Felício Verónica, Alves Paulo C, Vieira Isabel, Amaral Margarida D

Abstract excerpt

BACKGROUND: Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), the dysfunctional Cl(-) channel in Cystic Fibrosis, undergoes complex biosynthesis at the endoplasmic reticulum involving several molecular chaperones including Hsp70 and many co-chaperones. Bcl-2- associated athanogenes (BAGs) constitute a protein family sharing an Hsc70-binding domain. BAG-1 possesses an ubiquitin-like domain (Ub-LD)...

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Identifiers

PMID: 23178238
DOI: 10.1159/000343303

Genes

CFTR

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-like-domain-dependent manner.PMID 23178238Read ↗

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

The multiple ubiquitination mechanisms in CFTR peripheral quality control.Semantic proximity Read ↗

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Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.Semantic proximity Read ↗

Interference with ubiquitination in CFTR modifies stability of core glycosylated and cell surface pools.Semantic proximity Read ↗

Cystic fibrosis transmembrane conductance regulator degradation: cross-talk between the ubiquitylation and SUMOylation pathways.Semantic proximity Read ↗

Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants.Semantic proximity Read ↗

BAG-6 is essential for selective elimination of defective proteasomal substratesSemantic proximity Read ↗

CFTR and chaperones: processing and degradation.Semantic proximity Read ↗

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Degradation of CFTR-F508del By the Ubiquitin E2 Conjugating Enzyme UBE 2L6 and the E3 Ligase RNF19BSemantic proximity Read ↗

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