A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds
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Processing of pulmonary surfactant protein B by napsin and cathepsin HHuman pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expressionl7Rn6 encodes a novel protein required for clara cell function in mouse lung developmentGenetic interstitial lung diseaseCharacteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan.Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesisInterstitial lung disease in children -- genetic background and associated phenotypes.An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancyMetabolic Functions of the Lung, Disorders and Associated PathologiesCompound SFTPB 1549C-->GAA (121ins2) and 457delC heterozygosity in severe congenital lung disease and surfactant protein B (SP-B) deficiencyRetinoic acid stimulation of the human surfactant protein B promoter is thyroid transcription factor 1 site-dependentGenetic disorders of surfactant dysfunctionAlveolar surfactant homeostasis and the pathogenesis of pulmonary diseasePulmonary surfactant pathophysiology: current models and open questionsThe Pulmonary Surfactant: Impact of Tobacco Smoke and Related Compounds on Surfactant and Lung DevelopmentThe pulmonary surfactant: impact of tobacco smoke and related compounds on surfactant and lung developmentDeletion of STK40 protein in mice causes respiratory failure and death at birth.Lymphatic function is required prenatally for lung inflation at birth.Fgf10 dosage is critical for the amplification of epithelial cell progenitors and for the formation of multiple mesenchymal lineages during lung developmentLoss of Lysyl Oxidase-like 3 Attenuates Embryonic Lung Development in MiceEffects of the lung surfactant protein B construct Mini-B on lipid bilayer order and topographyRole of the N-terminal seven residues of surfactant protein B (SP-B)Involvement of napsin A in the C- and N-terminal processing of surfactant protein B in type-II pneumocytes of the human lung.Effects of gramicidin-A on the adsorption of phospholipids to the air-water interface.Interaction of the C-terminal peptide of pulmonary surfactant protein B (SP-B) with a bicellar lipid mixture containing anionic lipid.Differential effects of lysophosphatidylcholine on the adsorption of phospholipids to an air/water interfaceSurfactant protein B polymorphisms are associated with severe respiratory syncytial virus infection, but not with asthmaPepsinogen C proteolytic processing of surfactant protein B.Critical structural and functional roles for the N-terminal insertion sequence in surfactant protein B analogs.Surfactant protein B propeptide contains a saposin-like protein domain with antimicrobial activity at low pHSurfactant replacement therapy--room for improvement.Isolation and characterization of alveolar epithelial type II cells derived from mouse embryonic stem cells.Genetic risk factors associated with respiratory distress syndromeHydrophobic surfactant proteins induce a phosphatidylethanolamine to form cubic phasesLung surfactant protein A (SP-A) interactions with model lung surfactant lipids and an SP-B fragment.The molecular era of surfactant biology.Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice.Pulmonary alveolar proteinosis: an overview for internists and hospital physicians.Pulmonary alveolar proteinosis: progress in the first 44 years.Dimeric N-terminal segment of human surfactant protein B (dSP-B(1-25)) has enhanced surface properties compared to monomeric SP-B(1-25)
P2860
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P2860
A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds
description
1994 nî lūn-bûn
@nan
1994 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
1994 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
1994年の論文
@ja
1994年論文
@yue
1994年論文
@zh-hant
1994年論文
@zh-hk
1994年論文
@zh-mo
1994年論文
@zh-tw
1994年论文
@wuu
name
A mutation in the surfactant p ...... y disease in multiple kindreds
@ast
A mutation in the surfactant p ...... y disease in multiple kindreds
@en
A mutation in the surfactant p ...... y disease in multiple kindreds
@nl
type
label
A mutation in the surfactant p ...... y disease in multiple kindreds
@ast
A mutation in the surfactant p ...... y disease in multiple kindreds
@en
A mutation in the surfactant p ...... y disease in multiple kindreds
@nl
prefLabel
A mutation in the surfactant p ...... y disease in multiple kindreds
@ast
A mutation in the surfactant p ...... y disease in multiple kindreds
@en
A mutation in the surfactant p ...... y disease in multiple kindreds
@nl
P2093
P2860
P3181
P356
P1476
A mutation in the surfactant p ...... y disease in multiple kindreds
@en
P2093
A M Murphy
D E deMello
H R Colten
P2860
P304
P3181
P356
10.1172/JCI117173
P407
P577
1994-04-01T00:00:00Z