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Article

Chemical chaperones reduce endoplasmic reticulum stress and prevent mutant HFE aggregate formation.

The Journal of biological chemistry - 21 Sept 2007

de Almeida Sérgio F, Picarote Gonçalo, Fleming John V, Carmo-Fonseca Maria, Azevedo Jorge E, de Sousa Maria

Abstract excerpt

HFE C282Y, the mutant protein associated with hereditary hemochromatosis (HH), fails to acquire the correct conformation in the endoplasmic reticulum (ER) and is targeted for degradation. We have recently shown that an active unfolded protein response (UPR) is present in the cells of patients with HH. Now, by using HEK 293T cells, we demonstrate that the stability of HFE C282Y is influenced by the UPR signaling...

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Share to Endoplasmic Reticulum
Share to Hemochromatosis

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Share to Histocompatibility Antigens Class I

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Share to Phenylbutyrates

Share to Protein Denaturation

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Source

Identifiers

PMID: 17626021
DOI: 10.1074/jbc.M702672200

Genes

HFE

Variants

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.

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