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Mucus clearance as a primary innate defense mechanism for mammalian airways.
The conducting airways branch 20–25 times between the trachea and the alveoli as inhaled air passes from the relatively constricted nasal/tracheal passages to the large surface area of alveoli (70Expand
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Primary ciliary dyskinesia: diagnostic and phenotypic features.
Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormalities in ciliary structure/function. We hypothesized that the major clinical and biologic phenotypic markers of theExpand
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Mucus clearance and lung function in cystic fibrosis with hypertonic saline.
BACKGROUND Abnormal homeostasis of the volume of airway surface liquid in patients with cystic fibrosis is thought to produce defects in mucus clearance and airway defense. Through osmotic forces,Expand
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Nontuberculous mycobacteria. I: multicenter prevalence study in cystic fibrosis.
  • K. Olivier, David J Weber, +10 authors M. Knowles
  • Medicine
  • American journal of respiratory and critical care…
  • 15 March 2003
Nontuberculous mycobacteria (NTM) are potential respiratory pathogens in cystic fibrosis (CF). To assess the species-specific prevalence and risk factors for acquisition, we conducted a prospective,Expand
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Expression and pharmacological characterization of the human D3 dopamine receptor.
Binding of dopamine receptor ligands to human D2 and D3 receptors was characterized in Chinese hamster ovary (CHO) cells using the dopamine D2 receptor antagonist [125I] iodosulpiride. Only limitedExpand
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Primary ciliary dyskinesia. Recent advances in diagnostics, genetics, and characterization of clinical disease.
Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder of motile cilia that leads to oto-sino-pulmonary diseases and organ laterality defects in approximately 50% ofExpand
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Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome
Primary ciliary dyskinesia is a genetically heterogeneous disorder of motile cilia. Most of the disease-causing mutations identified to date involve the heavy (dynein axonemal heavy chain 5) orExpand
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Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
BACKGROUND It is unknown whether genetic factors predispose patients to idiopathic pancreatitis. In patients with cystic fibrosis, mutations of the cystic fibrosis transmembrane conductance regulatorExpand
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DYX1C1 is required for axonemal dynein assembly and ciliary motility
DYX1C1 has been associated with dyslexia and neuronal migration in the developing neocortex. Unexpectedly, we found that deleting exons 2–4 of Dyx1c1 in mice caused a phenotype resembling primaryExpand
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Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects.
To test whether a major contribution of airways epithelial ion transport to lung defense reflects the regulation of airway surface liquid (ASL) ionic composition, we measured ASL composition usingExpand
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