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Article

Systematic review of drug effects in humans and models with surfactant-processing disease.

European respiratory review : an official journal of the European Respiratory Society - 30 Sept 2018

Klay Dymph, Hoffman Thijs W, Harmsze Ankie M, Grutters Jan C, van Moorsel Coline H M

Abstract excerpt

Fibrotic interstitial pneumonias are a group of rare diseases characterised by distortion of lung interstitium. Patients with mutations in surfactant-processing genes, such as surfactant protein C (SFTPC), surfactant protein A1 and A2 (SFTPA1 and A2), ATP binding cassette A3 (ABCA3) and Hermansky-Pudlak syndrome (HPS1, 2 and 4), develop progressive pulmonary fibrosis, often culminating in fatal respiratory...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 29997245
DOI: 10.1183/16000617.0135-2017
PMCID: PMC9489115

Genes

ABCA3
HPS1
SFTPA1
SFTPC

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 3 Sept 2026.

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