Nitric oxide and protein nitration in the cystic fibrosis airway☆
Section snippets
Subjects and specimens
For serum and exhaled NO measurements, subjects were recruited from a University-based outpatient adult CF center and from normal volunteers. The 18 CF patients carried various CFTR genotypes with delta-F508 and G551D predominant, in general reflecting the most common CFTR genotypes in the US CF population. All were self-described as having their baseline pulmonary symptoms and none were on systemic steroids. They were maintained on their standard regimens including standard CF nutritional
Results and discussion
The objective of this study was to develop a better understanding of the abnormalities of NO metabolism in the respiratory tract of patients with CF. As have most other investigators [15], [29] but not all [30], [31], we report a decreased exhaled NO in the CF patients studied (Table 1). The CF patients had normal serum levels of the NOS substrate l-arginine, increased serum NO2−/NO3− levels, subnormal levels of NOS2 protein in airway epithelial cells but abundant NOS2 protein in airway
Acknowledgements
We thank Ms. Julie Harral for technical assistance in performance of immunohistochemical studies, Ms. Cheryl Oliver-Pickett for assistance in the serum NOx measurements, and Drs. A. van der Vliet, C.E. Cross, and J.P. Eiserich for helpful suggestions and manuscript review.
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These studies were supported by a Clinical Fellowship Award from the Cystic Fibrosis Foundation (B.M.M.) and NIH HL48038 (D.M.R.).