RGD Reference Report - Increased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycin. - Rat Genome Database

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Increased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycin.

Authors: Lawson, WE  Polosukhin, VV  Stathopoulos, GT  Zoia, O  Han, W  Lane, KB  Li, B  Donnelly, EF  Holburn, GE  Lewis, KG  Collins, RD  Hull, WM  Glasser, SW  Whitsett, JA  Blackwell, TS 
Citation: Lawson WE, etal., Am J Pathol. 2005 Nov;167(5):1267-77.
RGD ID: 4144124
Pubmed: PMID:16251411   (View Abstract at PubMed)
PMCID: PMC1603790   (View Article at PubMed Central)
DOI: DOI:10.1016/S0002-9440(10)61214-X   (Journal Full-text)

Recent reports have linked mutations in the surfactant protein C gene (SFTPC) to familial forms of pulmonary fibrosis, but it is uncertain whether deficiency of mature SP-C contributes to disease pathogenesis. In this study, we evaluated bleomycin-induced lung fibrosis in mice with genetic deletion of SFTPC. Compared with wild-type (SFTPC+/+) controls, mice lacking surfactant protein C (SFTPC-/-) had greater lung neutrophil influx at 1 week after intratracheal bleomycin, greater weight loss during the first 2 weeks, and increased mortality. At 3 and 6 weeks after bleomycin, lungs from SFTPC-/- mice had increased fibroblast numbers, augmented collagen accumulation, and greater parenchymal distortion. Furthermore, resolution of fibrosis was delayed. Although remodeling was near complete in SFTPC+/+ mice by 6 weeks, SFTPC-/- mice did not return to baseline until 9 weeks after bleomycin. By terminal dUTP nick-end labeling staining, widespread cell injury was observed in SFTPC-/- and SFTPC+/+ mice 1 week after bleomycin; however, ongoing apoptosis of epithelial and interstitial cells occurred in lungs of SFTPC-/- mice, but not SFTPC+/+ mice, 6 weeks after bleomycin. Thus, SP-C functions to limit lung inflammation, inhibit collagen accumulation, and restore normal lung structure after bleomycin.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
pulmonary fibrosis  ISOSftpc (Mus musculus)4144124; 4144124 RGD 
pulmonary fibrosis  IMP 4144124 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Sftpc  (surfactant protein C)

Genes (Mus musculus)
Sftpc  (surfactant associated protein C)

Genes (Homo sapiens)
SFTPC  (surfactant protein C)


Additional Information