The Journal of Experimental Medicine
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Published online
doi:10.1084/jem.20082378
The Journal of Experimental Medicine, Vol. 205, No. 12, 2693-2697
The Rockefeller University Press, 0022-1007 $30.00
© Notarangelo et al.
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COMMENTARY

 Out of breath: GM-CSFR{alpha} mutations disrupt surfactant homeostasis

Luigi D. Notarangelo and Itai Pessach

L.D.N. is at The Manton Center for Orphan Disease Research, Division of Immunology, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115
I.P. is at Division of Immunology, Children's Hospital, Harvard Medical School, Boston, MA 02115 and The Talpiot Medical Leadership Program, Safra Children's Hospital, Sheba Medical Center, Tel-Hashomer, Israel 52621

CORRESPONDENCE L.G.D.: luigi.notarangelo{at}childrens.harvard.edu


ABSTRACT
Pulmonary alveolar proteinosis (PAP) is a rare disorder in which surfactant homeostasis in the lung is impaired, causing respiratory distress and, in severe cases, respiratory failure. Most cases of PAP are associated with the formation of autoantibodies against the cytokine granulocyte/macrophage colony-stimulating factor (GM-CSF), which is required for normal surfactant homeostasis and lung function. New studies now identify three patients in whom PAP was caused by mutations in the gene encoding the ligand-binding {alpha} chain of the GM-CSF receptor.

© 2008 Notarangelo and Pessach This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.jem.org/misc/terms.shtml). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/3.0/).


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