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Article

BiP expression is not increased by the accumulation of PiZ alpha 1-antitrypsin in the endoplasmic reticulum.

FEBS letters - 16 Jul 1990

Cresteil D, Ciccarelli E, Soni T, Alonso M A, Jacobs P, Bollen A, Alvarez F

Abstract excerpt

PiZ, a mutant human alpha 1-antitrypsin, is associated with liver and pulmonary disease and is characterized by defective secretion and accumulation of the protein in the endoplasmic reticulum. We tested the hypothesis that BiP (a protein that binds newly synthesized protein in the endoplasmic reticulum, prevents secretion of incorrectly folded protein, and solubilizes protein aggregates), could play a part in...

Topics

Animals
Blotting, Northern
Carrier Proteins
Cell Line
Endoplasmic Reticulum
Endoplasmic Reticulum Chaperone BiP
Heat-Shock Proteins
Humans
Liver
Liver Diseases
Lung Diseases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Molecular Chaperones

Mutation

Protein Conformation

RNA, Messenger

alpha 1-Antitrypsin

Source

Identifiers

PMID: 2379586
DOI: 10.1016/0014-5793(90)80944-e

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 29 Aug 2026. Prepared record publication-2379586-2026-08-29.1. Manifest publication-graph-cohort-2026-08-29.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 29 Aug 2026.

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BiP expression is not increased by the accumulation of PiZ alpha 1-antitrypsin in the endoplasmic reticulum.PMID 2379586Read ↗

Accumulation of the insoluble PiZ variant of human alpha 1-antitrypsin within the hepatic endoplasmic reticulum does not elevate the steady-state level of grp78/BiP.Semantic proximity Read ↗

Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.Semantic proximity Read ↗

PDZK1 is required for maintaining hepatic scavenger receptor, class B, type I (SR-BI) steady state levels but not its surface localization or function.Semantic proximity Read ↗

Expression of PiM-and PiZ-mutated forms of the human alpha 1-antitrypsin gene in transfected monkey COS1 cells.Semantic proximity Read ↗

Hepatic alpha 1-antitrypsin mRNA content in cirrhosis with normal and abnormal protease inhibitor phenotypes.Semantic proximity Read ↗

Identification of PiZ gene products in liver tissue by a monoclonal antibody specific for the Z mutant of alpha 1-antitrypsin.Semantic proximity Read ↗

The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.Semantic proximity Read ↗

Molecular Cloning and Expression of Lipid Transfer Inhibitor Protein Reveals Its Identity with Apolipoprotein FSemantic proximity Read ↗

Liver involvement in infants with PiSZ phenotype of alpha 1-antitrypsin deficiency.Semantic proximity Read ↗

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