Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice.

The Journal of biological chemistry - 26 Dec 2003

Bridges James P, Wert Susan E, Nogee Lawrence M, Weaver Timothy E

Abstract excerpt

Surfactant Protein C (SP-C) is a secreted transmembrane protein that is exclusively expressed by alveolar type II epithelial cells of the lung. SP-C associates with surfactant lipids to reduce surface tension within the alveolus, maintaining lung volume at end expiration. Mutations in the gene en...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Endoplasmic Reticulum Chaperone BiP
Share to Heat-Shock Proteins
Share to Intercellular Signaling Peptides and Proteins

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Lipid Metabolism

Share to Luciferases

Share to Lung Diseases

Share to Molecular Chaperones

Share to Pulmonary Surfactant-Associated Protein C

Source

Identifiers

PMID: 14525980
DOI: 10.1074/jbc.M309599200

Genes

SFTPC

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026. Prepared record publication-14525980-2026-09-01.1. Manifest publication-graph-cohort-2026-09-01.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 1 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice.PMID 14525980Read ↗

Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative.Semantic proximity Read ↗

Overexpression of surfactant protein-C mature peptide causes neonatal lethality in transgenic mice.Semantic proximity Read ↗

Rescue of SP-B knockout mice with a truncated SP-B proprotein. Function of the C-terminal propeptide.Semantic proximity Read ↗

Surfactant protein C mutation links postnatal type 2 cell dysfunction to adult disease.Semantic proximity Read ↗

Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene.Semantic proximity Read ↗

A novel surfactant protein C mutation resulting in aberrant protein processing and altered subcellular localization causes infantile interstitial lung disease.Semantic proximity Read ↗

Divergent Pathways of Surfactant Protein C Maturation for Disease-Associated IsoformsSemantic proximity Read ↗

Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C.Semantic proximity Read ↗

Lung-tropic dual AAV-SP-C and microRNA gene therapy attenuates lung injury in mutant Sftpc mice.Semantic proximity Read ↗

Multiple ways to die: delineation of the unfolded protein response and apoptosis induced by Surfactant Protein C BRICHOS mutants.Semantic proximity Read ↗

Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene.Semantic proximity Read ↗

A nonaggregating surfactant protein C mutant is misdirected to early endosomes and disrupts phospholipid recycling.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.