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Article

N-terminally extended surfactant protein (SP) C isolated from SP-B-deficient children has reduced surface activity and inhibited lipopolysaccharide binding.

Biochemistry - 6 Apr 2004

Li Jing, Ikegami Machiko, Na Cheng-Lun, Hamvas Aaron, Espinassous Quentin, Chaby Richard, Nogee Lawrence M, Weaver Timothy E, Johansson Jan

Abstract excerpt

In both humans and mice, a deficiency of surfactant protein B (SP-B) is associated with a decreased concentration of mature SP-C and accumulation of a larger SP-C peptide, denoted SP-C(i), which is not observed under normal conditions. Isolation of hydrophobic polypeptides from the lungs of children who died with two different SP-B mutations yielded pure SP-C(i) and showed only trace amounts of mature SP-C....

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Share to Bronchoalveolar Lavage Fluid
Share to Chromatography, High Pressure Liquid

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Share to Pulmonary Surfactant-Associated Protein B

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Share to Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

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Source

Identifiers

PMID: 15049696
DOI: 10.1021/bi036218q

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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