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Article

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

Journal of cell science - 15 Feb 2003

Wang Wen-Jing, Mulugeta Surafel, Russo Scott J, Beers Michael F

Abstract excerpt

Human surfactant protein C (hSP-C) is synthesized by the alveolar type 2 cell as a 197 amino acid integral membrane proprotein and proteolytically processed to a secreted 3.7 kDa mature form. Although the SP-C null mouse possesses a non-lethal phenotype, a heterozygous substitution of A for G in the first base of intron 4 of the human SP-C gene (c.460+1A>G) has been reported in association with familial...

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Identifiers

PMID: 12538769
DOI: 10.1242/jcs.00267

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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