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  1. Article ; Online: SARS-CoV-2 Infection of Airway Cells.

    Ehre, Camille

    The New England journal of medicine

    2020  Volume 383, Issue 10, Page(s) 969

    MeSH term(s) Betacoronavirus/ultrastructure ; Bronchi/cytology ; COVID-19 ; Cells, Cultured ; Coronavirus Infections/pathology ; Epithelial Cells/ultrastructure ; Epithelial Cells/virology ; Humans ; Microscopy, Electron, Scanning ; Pandemics ; Pneumonia, Viral/pathology ; SARS-CoV-2 ; Virus Cultivation
    Keywords covid19
    Language English
    Publishing date 2020-09-02
    Publishing country United States
    Document type Journal Article
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMicm2023328
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  2. Article ; Online: Accumulation de mucus - Le point de départ de la pathogenèse pulmonaire chez les patients atteints de mucoviscidose.

    Ehre, Camille

    Medecine sciences : M/S

    2020  Volume 35, Issue 12, Page(s) 1217–1220

    Title translation Mucus buildup: the starting point of cystic fibrosis lung disease pathogenesis.
    MeSH term(s) Bronchiectasis/etiology ; Bronchiectasis/metabolism ; Bronchiectasis/pathology ; Child ; Child, Preschool ; Cilia/pathology ; Cilia/physiology ; Cystic Fibrosis/etiology ; Cystic Fibrosis/genetics ; Cystic Fibrosis/metabolism ; Cystic Fibrosis/pathology ; Cystic Fibrosis Transmembrane Conductance Regulator/physiology ; Disease Progression ; Humans ; Infant ; Lung/metabolism ; Lung/pathology ; Mucus/metabolism ; Protein Aggregation, Pathological/complications ; Protein Aggregation, Pathological/genetics ; Protein Aggregation, Pathological/metabolism ; Protein Aggregation, Pathological/pathology ; Water/metabolism
    Chemical Substances CFTR protein, human ; Water (059QF0KO0R) ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language French
    Publishing date 2020-01-06
    Publishing country France
    Document type News
    ZDB-ID 632733-3
    ISSN 1958-5381 ; 0767-0974
    ISSN (online) 1958-5381
    ISSN 0767-0974
    DOI 10.1051/medsci/2019234
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  4. Book ; Online: LOS TÉNTACULOS DE LA VIDA

    Ehre, Camille

    Los Ojos de la Ciencia; Vol 1, No

    2020  Volume 10

    Abstract: El efecto del coronavirus sobre las células pulmonares. Imagen de aumento de mayor potencia (microscopía electrónica de barrido) muestra la estructura y densidad de los viriones del SARS-CoV-2 (rojo) producidos por el epitelio de las vías respiratorias ... ...

    Abstract El efecto del coronavirus sobre las células pulmonares. Imagen de aumento de mayor potencia (microscopía electrónica de barrido) muestra la estructura y densidad de los viriones del SARS-CoV-2 (rojo) producidos por el epitelio de las vías respiratorias humanas. N Engl J Med 2020; 383:969 DOI:10.1056/NEJMicm2023328
    Keywords covid19
    Language Spanish
    Publishing date 2020-10-23
    Publisher Instituto Nacional de Higiene “Rafael Rangel”
    Publishing country ve
    Document type Book ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  5. Article: Un nouveau concept de mécanisme de clairance respiratoire ?

    Ehré, Camille

    Medecine sciences : M/S

    2013  Volume 29, Issue 2, Page(s) 144–146

    Title translation Mucus clearance in the respiratory tract: a new concept?.
    MeSH term(s) Body Fluids/metabolism ; Concept Formation ; Gels/metabolism ; Humans ; Models, Biological ; Mucociliary Clearance/genetics ; Mucociliary Clearance/physiology ; Mucus/chemistry ; Mucus/metabolism ; Mucus/secretion ; Pulmonary Disease, Chronic Obstructive/etiology ; Pulmonary Disease, Chronic Obstructive/metabolism ; Pulmonary Disease, Chronic Obstructive/pathology ; Respiratory System/metabolism ; Respiratory System/pathology
    Chemical Substances Gels
    Language French
    Publishing date 2013-02
    Publishing country France
    Document type News
    ZDB-ID 632733-3
    ISSN 1958-5381 ; 0767-0974
    ISSN (online) 1958-5381
    ISSN 0767-0974
    DOI 10.1051/medsci/2013292010
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  6. Article ; Online: Mucins and CFTR: Their Close Relationship.

    Okuda, Kenichi / Shaffer, Kendall M / Ehre, Camille

    International journal of molecular sciences

    2022  Volume 23, Issue 18

    Abstract: Mucociliary clearance is a critical defense mechanism for the lungs governed by regionally coordinated epithelial cellular activities, including mucin secretion, cilia beating, and transepithelial ion transport. Cystic fibrosis (CF), an autosomal genetic ...

    Abstract Mucociliary clearance is a critical defense mechanism for the lungs governed by regionally coordinated epithelial cellular activities, including mucin secretion, cilia beating, and transepithelial ion transport. Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) channel, is characterized by failed mucociliary clearance due to abnormal mucus biophysical properties. In recent years, with the development of highly effective modulator therapies, the quality of life of a significant number of people living with CF has greatly improved; however, further understanding the cellular biology relevant to CFTR and airway mucus biochemical interactions are necessary to develop novel therapies aimed at restoring
    MeSH term(s) Cystic Fibrosis/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; Mucociliary Clearance ; Mucus/metabolism ; Quality of Life
    Chemical Substances CFTR protein, human ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language English
    Publishing date 2022-09-06
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms231810232
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  7. Article ; Online: Mucus aberrant properties in CF: Insights from cells and animal models.

    Ehre, Camille / Hansson, Gunnar C / Thornton, David J / Ostedgaard, Lynda S

    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

    2022  Volume 22 Suppl 1, Page(s) S23–S26

    Abstract: Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus accumulation in the lungs, the intestinal tract, and the pancreatic ducts. ... ...

    Abstract Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus accumulation in the lungs, the intestinal tract, and the pancreatic ducts. Mucins are high-molecular-weight glycoproteins that govern the biochemical and biophysical properties of mucus. In the CF lung, increased mucus viscoelasticity is associated with decreased mucociliary clearance and defects in host defense mechanisms. The link between defective ion channel and abnormal mucus properties has been investigated in studies involving cell and animal models. In this review article, we discuss recent progress toward understanding the different regions and cells that express CFTR in the airways and how mucus is produced and cleared from the lungs. In addition, we reflect on animal models that provided insights into the organization and the role of the mucin network and how mucus and antimicrobial activities act in concert to protect the lungs from invading pathogens.
    MeSH term(s) Animals ; Cystic Fibrosis ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Mucus/metabolism ; Mucins/metabolism ; Lung ; Models, Animal
    Chemical Substances Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) ; Mucins
    Language English
    Publishing date 2022-09-16
    Publishing country Netherlands
    Document type Journal Article ; Review ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 2084724-5
    ISSN 1873-5010 ; 1569-1993
    ISSN (online) 1873-5010
    ISSN 1569-1993
    DOI 10.1016/j.jcf.2022.08.019
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  8. Article ; Online: Mucus, mucins, and cystic fibrosis.

    Morrison, Cameron Bradley / Markovetz, Matthew Raymond / Ehre, Camille

    Pediatric pulmonology

    2019  Volume 54 Suppl 3, Page(s) S84–S96

    Abstract: Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and is characterized by the accumulation of thick, ... ...

    Abstract Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and is characterized by the accumulation of thick, adherent mucus plaques in multiple organs, of which the lungs, gastrointestinal tract and pancreatic ducts are the most commonly affected. A similar pathogenesis cascade is observed in all of these organs: loss of CFTR function leads to altered ion transport, consisting of decreased chloride and bicarbonate secretion via the CFTR channel and increased sodium absorption via epithelial sodium channel upregulation. Mucosa exposed to changes in ionic concentrations sustain severe pathophysiological consequences. Altered mucus biophysical properties and weakened innate defense mechanisms ensue, furthering the progression of the disease. Mucins, the high-molecular-weight glycoproteins responsible for the viscoelastic properties of the mucus, play a key role in the disease but the actual mechanism of mucus accumulation is still undetermined. Multiple hypotheses regarding the impact of CFTR malfunction on mucus have been proposed and are reviewed here. (a) Dehydration increases mucin monomer entanglement, (b) defective Ca
    MeSH term(s) Cystic Fibrosis/physiopathology ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Humans ; Mucins/metabolism ; Mucus/metabolism
    Chemical Substances CFTR protein, human ; Mucins ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6)
    Language English
    Publishing date 2019-11-08
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 632784-9
    ISSN 1099-0496 ; 8755-6863
    ISSN (online) 1099-0496
    ISSN 8755-6863
    DOI 10.1002/ppul.24530
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    Zs.A 2143: Show issues Location:
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  9. Article ; Online: BPIFB1 loss alters airway mucus properties and diminishes mucociliary clearance.

    Donoghue, Lauren J / Markovetz, Matthew R / Morrison, Cameron B / Chen, Gang / McFadden, Kathryn M / Sadritabrizi, Taraneh / Gutay, Mark I / Kato, Takafumi / Rogers, Troy D / Snead, Jazmin Y / Livraghi-Butrico, Alessandra / Button, Brian / Ehre, Camille / Grubb, Barbara R / Hill, David B / Kelada, Samir N P

    American journal of physiology. Lung cellular and molecular physiology

    2023  Volume 325, Issue 6, Page(s) L765–L775

    Abstract: Airway mucociliary clearance (MCC) is required for host defense and is often diminished in chronic lung diseases. Effective clearance depends upon coordinated actions of the airway epithelium and a mobile mucus layer. Dysregulation of the primary ... ...

    Abstract Airway mucociliary clearance (MCC) is required for host defense and is often diminished in chronic lung diseases. Effective clearance depends upon coordinated actions of the airway epithelium and a mobile mucus layer. Dysregulation of the primary secreted airway mucin proteins, MUC5B and MUC5AC, is associated with a reduction in the rate of MCC; however, how other secreted proteins impact the integrity of the mucus layer and MCC remains unclear. We previously identified the gene
    MeSH term(s) Mice ; Humans ; Animals ; Mucociliary Clearance/physiology ; Respiratory System/metabolism ; Mucus/metabolism ; Lung Diseases/metabolism ; Mice, Knockout
    Language English
    Publishing date 2023-10-17
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Extramural
    ZDB-ID 1013184-x
    ISSN 1522-1504 ; 1040-0605
    ISSN (online) 1522-1504
    ISSN 1040-0605
    DOI 10.1152/ajplung.00390.2022
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  10. Article ; Online: Biochemical and rheological analysis of human colonic culture mucus reveals similarity to gut mucus.

    Howard, R Logan / Markovetz, Matthew / Wang, Yuli / Ehre, Camille / Sheikh, Shehzad Z / Allbritton, Nancy L / Hill, David B

    Biophysical journal

    2021  Volume 120, Issue 23, Page(s) 5384–5394

    Abstract: The goal of this project was to validate the functional relevance and utility of mucus produced by an in vitro intestinal cell culture model. This is facilitated by the need to physiologically replicate both healthy and abnormal mucus conditions from ... ...

    Abstract The goal of this project was to validate the functional relevance and utility of mucus produced by an in vitro intestinal cell culture model. This is facilitated by the need to physiologically replicate both healthy and abnormal mucus conditions from native intestinal tissue, where mucus properties have been connected to intestinal disease models. Mucus harvested from colonic cell cultures derived from healthy donors was compared to mucus collected from surgically resected, noninflamed transverse colon tissue. The rheological and biochemical properties of these mucus samples were compared using oscillational rheometry, particle-tracking microrheology, multiangle laser light scattering, refractometry, and immunohistochemical imaging. An air-liquid interface culture of primary human colonic epithelial cells generated a continuous monolayer with an attached mucus layer that displayed increasing weight percent (wt%) of solids over 1 week (1.3 ± 0.5% at 2 days vs. 2.4 ± 0.3% at 7 days). The full range of mucus concentrations (0.9-3.3%) observed during culture was comparable to that displayed by ex vivo mucus (1.3-1.9%). Bulk rheological measurements displayed similar wt%-based complex viscosities between in vitro and ex vivo mucus, with the complex viscosity of both systems increasing with wt% of solids. Particle-tracking microrheology showed higher complex viscosities for ex vivo mucus samples than in vitro mucus which was explained by a greater fraction of water present in in vitro mucus than ex vivo, i.e., in vitro mucus is more heterogeneous than ex vivo. Refractometry, multiangle laser light scattering, and immunostaining showed increased mucus complex size in ex vivo mucus compared with in vitro mucus, which may have been due to the admixture of mucus and cellular debris during ex vivo mucus collection. The air-liquid interface culture system produced intestinal mucus with similar composition and rheology to native human gut mucus, providing a platform to analyze pathological differences in intestinal mucus.
    MeSH term(s) Humans ; Intestinal Mucosa ; Intestines ; Mucus ; Rheology ; Viscosity
    Language English
    Publishing date 2021-10-23
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 218078-9
    ISSN 1542-0086 ; 0006-3495
    ISSN (online) 1542-0086
    ISSN 0006-3495
    DOI 10.1016/j.bpj.2021.10.024
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