Mutations in RSPH1 Cause Primary Ciliary Dyskinesia with a Unique Clinical and Ciliary Phenotype
…, LE Ostrowski, MW Leigh, PR Sears… - American journal of …, 2014 - atsjournals.org
Rationale: Primary ciliary dyskinesia (PCD) is a genetically heterogeneous recessive disorder
of motile cilia, but the genetic cause is not defined for all patients with PCD. Objectives: To …
of motile cilia, but the genetic cause is not defined for all patients with PCD. Objectives: To …
[PDF][PDF] Lack of GAS2L2 causes PCD by impairing cilia orientation and mucociliary clearance
XM Bustamante-Marin, WN Yin, PR Sears… - The American Journal of …, 2019 - cell.com
Primary ciliary dyskinesia (PCD) is a genetic disorder in which impaired ciliary function
leads to chronic airway disease. Exome sequencing of a PCD subject identified an apparent …
leads to chronic airway disease. Exome sequencing of a PCD subject identified an apparent …
Continuous mucociliary transport by primary human airway epithelial cells in vitro
PR Sears, WN Yin… - American Journal of …, 2015 - journals.physiology.org
Mucociliary clearance (MCC) is an important innate defense mechanism that continuously
removes inhaled pathogens and particulates from the airways. Normal MCC is essential for …
removes inhaled pathogens and particulates from the airways. Normal MCC is essential for …
A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance
…, MA Zariwala, J Carson, MLA Daniels, PR Sears… - Nature medicine, 2020 - nature.com
Mucociliary clearance, the physiological process by which mammalian conducting airways
expel pathogens and unwanted surface materials from the respiratory tract, depends on the …
expel pathogens and unwanted surface materials from the respiratory tract, depends on the …
[HTML][HTML] Mutation of CFAP57, a protein required for the asymmetric targeting of a subset of inner dynein arms in Chlamydomonas, causes primary ciliary dyskinesia
Primary ciliary dyskinesia (PCD) is characterized by chronic airway disease, reduced fertility,
and randomization of the left/right body axis. It is caused by defects of motile cilia and …
and randomization of the left/right body axis. It is caused by defects of motile cilia and …
Identification of genetic variants in CFAP221 as a cause of primary ciliary dyskinesia
XM Bustamante-Marin, A Shapiro, PR Sears… - Journal of human …, 2020 - nature.com
Primary ciliary dyskinesia (PCD) is a rare disorder that affects the biogenesis or function of
motile cilia resulting in chronic airway disease. PCD is genetically and phenotypically …
motile cilia resulting in chronic airway disease. PCD is genetically and phenotypically …
Mucociliary interactions and mucus dynamics in ciliated human bronchial epithelial cell cultures
PR Sears, CW Davis, M Chua… - American Journal of …, 2011 - journals.physiology.org
The airway epithelial surface liquid is generally considered to be composed of two layers, a
periciliary layer and a continuous thick mucus layer moving in bulk. This view may not be …
periciliary layer and a continuous thick mucus layer moving in bulk. This view may not be …
[HTML][HTML] Sperm-associated antigen 6 (SPAG6) deficiency and defects in ciliogenesis and cilia function: polarity, density, and beat
SPAG6, an axoneme central apparatus protein, is essential for function of ependymal cell
cilia and sperm flagella. A significant number of Spag6-deficient mice die with hydrocephalus, …
cilia and sperm flagella. A significant number of Spag6-deficient mice die with hydrocephalus, …
[PDF][PDF] Induction of ciliary orientation by matrix patterning and characterization of mucociliary transport
PR Sears, XM Bustamante-Marin, H Gong… - Biophysical …, 2021 - cell.com
Impaired mucociliary clearance (MCC) is a key feature of many airway diseases, including
asthma, bronchiectasis, chronic obstructive pulmonary disease, cystic fibrosis, and primary …
asthma, bronchiectasis, chronic obstructive pulmonary disease, cystic fibrosis, and primary …
High-speed and high-sensitivity parallel spectral-domain optical coherence tomography using a supercontinuum light source
J Barrick, A Doblas, MR Gardner, PR Sears… - Optics letters, 2016 - opg.optica.org
The three most important metrics in optical coherence tomography (OCT) are resolution, speed,
and sensitivity. Because there is a complex interplay between these metrics, no previous …
and sensitivity. Because there is a complex interplay between these metrics, no previous …