American Journal of Respiratory and Critical Care Medicine Vol 165. pp. 1322-1328, (2002)
© 2002 American Thoracic Society
Heterozygosity for a Surfactant Protein C Gene Mutation Associated with Usual Interstitial Pneumonitis and Cellular Nonspecific Interstitial Pneumonitis in One Kindred
Alan Q. Thomas,
Kirk Lane,
John Phillips, III,
Melissa Prince,
Cheryl Markin,
Marcy Speer,
David A. Schwartz,
Radhika Gaddipati,
Annis Marney,
Joyce Johnson,
Richard Roberts,
Jonathan Haines,
Mildred Stahlman and
James E. Loyd
Division of Allergy, Pulmonary and Critical Care Medicine; Division of Medical Genetics; Department of Pathology; Program in Human Genetics; Department of Pediatrics, Vanderbilt University Medical Center, Nashville; and Division of Pulmonary and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina
Correspondence and requests for reprints should be addressed to Alan Q. Thomas, M.D., Center for Lung Research, Vanderbilt University Medical Center, T-1217 Medical Center North, Nashville, TN 37232-2650. E-mail: alan.thomas{at}mcmail.vanderbilt.edu
Familial pulmonary fibrosis is a heterogeneous group of interstitial lung diseases of unknown cause that is associated with multiple pathologic subsets. Mutations in the surfactant protein C (SP-C) gene (SFTPC) are associated with familial desquamative and nonspecific interstitial pneumonitis. Genetic studies in familial usual interstitial pneumonitis have been inconclusive. Using a candidate gene approach, we found a heterozygous exon 5 + 128 T A transversion of SFTPC in a large familial pulmonary fibrosis kindred, including adults with usual interstitial pneumonitis and children with cellular nonspecific interstitial pneumonitis. The mutation is predicted to substitute a glutamine for a conserved leucine residue and may hinder processing of SP-C precursor protein. SP-C precursor protein displayed aberrant subcellular localization by immunostaining. Electron microscopy of affected lung revealed alveolar type II cell atypia, with numerous abnormal lamellar bodies. Mouse lung epithelial cells transfected with the SFTPC mutation were notable for similar electron microscopy findings and for exaggerated cellular toxicity. We show that an SFTPC mutation segregates with the pulmonary fibrosis phenotype in this kindred and may cause type II cellular injury. The presence of two different pathologic diagnoses in affected relatives sharing this mutation indicates that in this kindred, these diseases may represent pleiotropic manifestations of the same central pathogenesis.
Key Words: pulmonary fibrosis • familial • surfactant protein C • interstitial lung disease • genetics
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Acute Respiratory Failure after Interferon-{gamma} Therapy of End-Stage Pulmonary Fibrosis
Am. J. Respir. Crit. Care Med.,
April 1, 2003;
167(7):
953 - 957.
[Abstract]
[Full Text]
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W.-J. Wang, S. Mulugeta, S. J. Russo, and M. F. Beers
Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative
J. Cell Sci.,
February 15, 2003;
116(4):
683 - 692.
[Abstract]
[Full Text]
[PDF]
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M. J. Tobin
Pediatrics, Surfactant, and Cystic Fibrosis in AJRCCM 2002
Am. J. Respir. Crit. Care Med.,
February 1, 2003;
167(3):
333 - 344.
[Full Text]
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M. J. Tobin
Tuberculosis, Lung Infections, Interstitial Lung Disease, and Journalology in AJRCCM 2002
Am. J. Respir. Crit. Care Med.,
February 1, 2003;
167(3):
345 - 355.
[Full Text]
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J. A. Whitsett and T. E. Weaver
Hydrophobic Surfactant Proteins in Lung Function and Disease
N. Engl. J. Med.,
December 26, 2002;
347(26):
2141 - 2148.
[Full Text]
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J. A. Whitsett
Genetic Basis of Familial Interstitial Lung Disease: Misfolding or Function of Surfactant Protein C?
Am. J. Respir. Crit. Care Med.,
May 1, 2002;
165(9):
1201 - 1202.
[Full Text]
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Copyright © 2002 American Thoracic Society
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