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Article

Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells.

Respiratory research - 7 Jan 2011

Weichert Nina, Kaltenborn Eva, Hector Andreas, Woischnik Markus, Schams Andrea, Holzinger Andreas, Kern Sunčana, Griese Matthias

Abstract excerpt

BACKGROUND: ABCA3 transporter (ATP-binding cassette transporter of the A subfamily) is localized to the limiting membrane of lamellar bodies, organelles for assembly and storage of pulmonary surfactant in alveolar epithelial type II cells (AECII). It transports surfactant phospholipids into lamellar bodies and absence of ABCA3 function disrupts lamellar body biogenesis. Mutations of the ABCA3 gene lead to fatal...

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Source

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Identifiers

PMID: 21214890
DOI: 10.1186/1465-9921-12-4
PMCID: PMC3024939

Genes

ABCA3
XBP1

Variants

Provenance

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.

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Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells.PMID 21214890Read ↗

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ABCA3 is critical for lamellar body biogenesis in vivo.Semantic proximity Read ↗

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