A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance.

Adolescent Adult Cell Separation Child Ciliopathies / immunology Cystic Fibrosis Transmembrane Conductance Regulator / metabolism Epithelial Cells / metabolism Exome Female Flow Cytometry HEK293 Cells Homozygote Humans Microscopy, Phase-Contrast Microscopy, Video Mucociliary Clearance Mutation NIMA-Related Kinases / metabolism Phenotype Proteome Respiratory System Tomography, X-Ray Computed X-Ray Microtomography Young Adult

Journal

Nature medicine
ISSN: 1546-170X
Titre abrégé: Nat Med
Pays: United States
ID NLM: 9502015

Informations de publication

Date de publication:
02 2020
Historique:
received: 11 04 2019
accepted: 06 12 2019
pubmed: 22 1 2020
medline: 22 4 2020
entrez: 22 1 2020
Statut: ppublish

Résumé

Mucociliary clearance, the physiological process by which mammalian conducting airways expel pathogens and unwanted surface materials from the respiratory tract, depends on the coordinated function of multiple specialized cell types, including basal stem cells, mucus-secreting goblet cells, motile ciliated cells, cystic fibrosis transmembrane conductance regulator (CFTR)-rich ionocytes, and immune cells

Identifiants

DOI: 10.1038/s41591-019-0730-x PMID: 31959991 PMC: PMC7018620
pubmed: 31959991
doi: 10.1038/s41591-019-0730-x
pii: 10.1038/s41591-019-0730-x
pmc: PMC7018620
mid: NIHMS1546020
doi:

Substances chimiques

CFTR protein, human 0
Proteome 0
Cystic Fibrosis Transmembrane Conductance Regulator 126880-72-6
NIMA-Related Kinases EC 2.7.11.1
Nek10 protein, human EC 2.7.11.1

Types de publication

Case Reports Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

244-251

Subventions

Organisme : NCATS NIH HHS
ID : U2C TR002818
Pays : United States
Organisme : NHLBI NIH HHS
ID : U54 HL096458
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL117836
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL071798
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR000083
Pays : United States
Organisme : NIH HHS
ID : S10 OD012027
Pays : United States
Organisme : NHLBI NIH HHS
ID : K08 HL133603
Pays : United States
Organisme : NIAID NIH HHS
ID : R01 AI047389
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA103866
Pays : United States
Organisme : NCI NIH HHS
ID : T32 CA009216
Pays : United States
Organisme : NIAID NIH HHS
ID : R37 AI047389
Pays : United States
Organisme : NHLBI NIH HHS
ID : T32 HL116275
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA129105
Pays : United States

Commentaires et corrections

Type : ErratumIn

Références

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Auteurs

Raghu R Chivukula (RR)

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA. raghu@wi.mit.edu.
Whitehead Institute for Biomedical Research, Cambridge, MA, USA. raghu@wi.mit.edu.
Howard Hughes Medical Institute, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA. raghu@wi.mit.edu.
Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, MA, USA. raghu@wi.mit.edu.
Koch Institute for Integrative Cancer Research, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA. raghu@wi.mit.edu.
ORCID: 0000-0001-5264-3196

Daniel T Montoro (DT)

Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, MA, USA.

Hui Min Leung (HM)

Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA, USA.
Harvard Medical School, Boston, MA, USA.

Jason Yang (J)

Whitehead Institute for Biomedical Research, Cambridge, MA, USA.
Howard Hughes Medical Institute, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.
Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, MA, USA.
Koch Institute for Integrative Cancer Research, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.

Hanan E Shamseldin (HE)

Department of Genetics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Martin S Taylor (MS)

Whitehead Institute for Biomedical Research, Cambridge, MA, USA.
Howard Hughes Medical Institute, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.
Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, MA, USA.
Koch Institute for Integrative Cancer Research, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.
Department of Pathology, Massachusetts General Hospital, Boston, MA, USA.

Gerard W Dougherty (GW)

Department of General Pediatrics, University Children's Hospital Muenster, Münster, Germany.

Maimoona A Zariwala (MA)

Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Johnny Carson (J)

Department of Pediatrics, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

M Leigh Anne Daniels (MLA)

Division of Pulmonary Diseases and Critical Care Medicine, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
ORCID: 0000-0001-6979-2681

Patrick R Sears (PR)

Cystic Fibrosis/Pulmonary Research and Treatment Center, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Katharine E Black (KE)

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.

Lida P Hariri (LP)

Department of Pathology, Massachusetts General Hospital, Boston, MA, USA.

Ibrahim Almogarri (I)

Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Evgeni M Frenkel (EM)

Whitehead Institute for Biomedical Research, Cambridge, MA, USA.
Howard Hughes Medical Institute, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.
Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, MA, USA.
Koch Institute for Integrative Cancer Research, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.

Vladimir Vinarsky (V)

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.

Heymut Omran (H)

Department of General Pediatrics, University Children's Hospital Muenster, Münster, Germany.

Michael R Knowles (MR)

Cystic Fibrosis/Pulmonary Research and Treatment Center, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Guillermo J Tearney (GJ)

Wellman Center for Photomedicine, Massachusetts General Hospital, Boston, MA, USA.
Harvard Medical School, Boston, MA, USA.
Department of Pathology, Massachusetts General Hospital, Boston, MA, USA.
Harvard-MIT Division of Health Sciences and Technology, Cambridge, MA, USA.

Fowzan S Alkuraya (FS)

Department of Genetics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. falkuraya@kfshrc.edu.sa.
ORCID: 0000-0003-4158-341X

David M Sabatini (DM)

Whitehead Institute for Biomedical Research, Cambridge, MA, USA.
Howard Hughes Medical Institute, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.
Broad Institute of Massachusetts Institute of Technology and Harvard, Cambridge, MA, USA.
Koch Institute for Integrative Cancer Research, Department of Biology, Massachusetts Institute of Technology, Cambridge, MA, USA.
ORCID: 0000-0002-1446-7256

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adolescent › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Techniques d'investigation › Techniques cytologiques › Séparation cellulaire › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies génétiques congénitales › Ciliopathies › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines membranaires › Protéines de transport membranaire › Pompes ioniques › Transporteurs d'anions › Transporteurs d'anions organiques › Transporteurs d'anions organiques ATP-dépendants › Protéines associées à la multirésistance aux médicaments › Protéine CFTR › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Cellules › Cellules épithéliales › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Phénomènes génétiques › Structures génétiques › Génome › Exome › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Techniques d'investigation › Techniques cytologiques › Cytophotométrie › Cytométrie en flux › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Cellules › Cellules épithéliales › Cellules HEK293 › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Phénomènes génétiques › Génotype › Homozygote › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Techniques d'investigation › Microscopie interférentielle › Microscopie de contraste de phase › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Sciences de l'information › Sources d'information › Moyens de communication › Télécommunications › Télévision › Vidéomicroscopie › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Phénomènes physiologiques respiratoires et circulatoires › Phénomènes physiologiques respiratoires › Clairance mucociliaire › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Phénomènes génétiques › Variation génétique › Mutation › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéines et peptides de signalisation intracellulaire › Protein-Serine-Threonine Kinases › Kinases apparentées à NIMA › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Phénomènes génétiques › Phénotype › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Protéome › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Appareil respiratoire › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Diagnostic › Techniques et procédures diagnostiques › Imagerie diagnostique › Tomographie › Tomographie à rayons X › Tomodensitométrie › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Diagnostic › Techniques et procédures diagnostiques › Imagerie diagnostique › Tomographie › Tomographie à rayons X › Tomodensitométrie › Microtomographie aux rayons X › A human ciliopathy reveals essential functions...
questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Jeune adulte › A human ciliopathy reveals essential functions...