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Complex two-gene modulation of lung disease...
Journal article

Complex two-gene modulation of lung disease severity in children with cystic fibrosis

Abstract

Although cystic fibrosis (CF) is a monogenic disease, its clinical manifestations are influenced in a complex manner. Severity of lung disease, the main cause of mortality among CF patients, is likely modulated by several genes. The mannose-binding lectin 2 (MBL2) gene encodes an innate immune response protein and has been implicated as a pulmonary modifier in CF. However, reports have been conflicting, and interactions with other modifiers …

Authors

Dorfman R; Sandford A; Taylor C; Huang B; Frangolias D; Wang Y; Sang R; Pereira L; Sun L; Berthiaume Y

Journal

Journal of Clinical Investigation, Vol. 118, No. 3, pp. 1040–1049

Publisher

American Society for Clinical Investigation

Publication Date

March 2008

DOI

10.1172/jci33754

ISSN

0021-9738