Cystic fibrosis and other respiratory diseases of impaired mucus clearance (Q36746482)

16 August 2017

1 reference

title

Cystic fibrosis and other respiratory diseases of impaired mucus clearance (English)

1 reference

16 August 2017

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Alessandra Livraghi

1

1 reference

PubMed ID

17325980

16 August 2017

Scott H Randell

series ordinal

2

1 reference

PubMed ID

17325980

16 August 2017

language of work or name

English

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publication date

1 January 2007

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16 August 2017

Toxicologic Pathology

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16 August 2017

35

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16 August 2017

1

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16 August 2017

116-129

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A Human Syndrome Caused by Immotile Cilia

16 August 2017

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

21 January 2018

Cilia-related diseases

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

The Ciliopathies: An Emerging Class of Human Genetic Disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Mutations in the DNAH11 (axonemal heavy chain dynein type 11) gene cause one form of situs inversus totalis and most likely primary ciliary dyskinesia

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infected lung in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Ciliogenesis and left-right axis defects in forkhead factor HFH-4-null mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Ultrastructural nasal pathology in children chronically and sequentially exposed to air pollutants

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

21 January 2018

The physiology of cough

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Mutation of the mouse hepatocyte nuclear factor/forkhead homologue 4 gene results in an absence of cilia and random left-right asymmetry

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

An incredible decade for the primary cilium: a look at a once-forgotten organelle

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Cystic fibrosis since 1938.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Familial immotile-cilia syndrome in English springer spaniel dogs.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Mislocalization of DNAH5 and DNAH9 in respiratory cells from patients with primary ciliary dyskinesia

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Novel tools to unravel molecular mechanisms in cilia-related disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Antimicrobial polypeptides in host defense of the respiratory tract

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Pathophysiology of gene-targeted mouse models for cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Anomalies in ion transport in CF mouse tracheal epithelium

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Nodal flow and the generation of left-right asymmetry.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

The nature of small-airway obstruction in chronic obstructive pulmonary disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

DNAH5 mutations are a common cause of primary ciliary dyskinesia with outer dynein arm defects

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Loss of function of axonemal dynein Mdnah5 causes primary ciliary dyskinesia and hydrocephalus

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

To beat or not to beat: roles of cilia in development and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Cystic fibrosis and airway submucosal glands

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Lung disease in mice with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Mucus clearance as a primary innate defense mechanism for mammalian airways

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Hydrocephalus, situs inversus, chronic sinusitis, and male infertility in DNA polymerase lambda-deficient mice: possible implication for the pathogenesis of immotile cilia syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Inhibition of chloride secretion in human bronchial epithelial cells by cigarette smoke extract.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Nucleotide release provides a mechanism for airway surface liquid homeostasis

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Cloned ferrets produced by somatic cell nuclear transfer

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Innate immune response in CF airway epithelia: hyperinflammatory?

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

No deleterious mutations in the FOXJ1 (alias HFH-4) gene in patients with primary ciliary dyskinesia (PCD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Innate immunity in the lungs

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Loss of binding and entry of liposome-DNA complexes decreases transfection efficiency in differentiated airway epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Global physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Primary ciliary dyskinesia (PCD).

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Disruption of an inner arm dynein heavy chain gene results in asthenozoospermia and reduced ciliary beat frequency

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Primary ciliary dyskinesia: diagnostic and phenotypic features.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Mutations in DNAH5 cause primary ciliary dyskinesia and randomization of left-right asymmetry

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Effective mucus clearance is essential for respiratory health

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Respiratory tract mucin genes and mucin glycoproteins in health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Intraflagellar transport and cilium-based signaling

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Animal models of cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Assembly and motility of eukaryotic cilia and flagella. Lessons from Chlamydomonas reinhardtii

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Preservation of Tracheal Mucus by Nonaqueous Fixative

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

The primary cilium as the cell's antenna: signaling at a sensory organelle

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

An animal model for cystic fibrosis made by gene targeting

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Ciliary function and the role of cilia in clearance

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Regulation of airway surface liquid volume and mucus transport by active ion transport

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Regulation of normal and cystic fibrosis airway surface liquid volume by phasic shear stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

The CF salt controversy: in vivo observations and therapeutic approaches.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

From mucins to mucus: toward a more coherent understanding of this essential barrier

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Primary ciliary dyskinesia: clinical presentation, diagnosis and genetics

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Respiratory infections with Pseudomonas aeruginosa in children with cystic fibrosis: early detection by serology and assessment of risk factors

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Airway mucus: From production to secretion.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Submucosal glands and airway defense

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Further studies on knockout mice lacking a functional dynein heavy chain (MDHC7). 2. A developmental explanation for the asthenozoospermia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Innate immunity and the lung: defense at the interface between host and environment

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Mutations of DNAI1 in primary ciliary dyskinesia: evidence of founder effect in a common mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

Investigation of the possible role of a novel gene, DPCD, in primary ciliary dyskinesia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1080%2F01926230601060025

10.1080/01926230601060025

21 January 2018

Identifiers

DOI

1 reference

16 August 2017

1 reference

PubMed ID

17325980