Genetic disorders of surfactant dysfunction (Q28235256)

Europe PubMed Central

title

Genetic disorders of surfactant dysfunction (English)

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author name string

Susan E Wert

series ordinal

1

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Jeffrey A Whitsett

series ordinal

2

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Lawrence M Nogee

series ordinal

3

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language of work or name

English

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publication date

2009

1 reference

Pediatric and Developmental Pathology

published in

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volume

12

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issue

4

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page(s)

253-74

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cites work

ERdj4 and ERdj5 are required for endoplasmic reticulum-associated protein degradation of misfolded surfactant protein C

1 reference

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Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease.

20 March 2017

1 reference

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Interstitial lung disease in children -- genetic background and associated phenotypes

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

A surfactant protein C precursor protein BRICHOS domain mutation causes endoplasmic reticulum stress, proteasome dysfunction, and caspase 3 activation

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Alterations in SP-B and SP-C expression in neonatal lung disease

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Processing of pulmonary surfactant protein B by napsin and cathepsin H

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Expression and localization of lung surfactant protein A in human tissues

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Disorders of vesicles of lysosomal lineage: the Hermansky-Pudlak syndromes

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABCA3 is a lamellar body membrane protein in human lung alveolar type II cells

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Synthesis and post-translational processing of surfactant protein C

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Altered stability of pulmonary surfactant in SP-C-deficient mice

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

A mutation in the surfactant protein C gene associated with familial interstitial lung disease

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Function of surfactant proteins B and C

20 March 2017

2 references

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

20 March 2017

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Localization of lung surfactant protein D on mucosal surfaces in human tissues

6 April 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Increased metalloproteinase activity, oxidant production, and emphysema in surfactant protein D gene-inactivated mice

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Intracellular localization of processing events in human surfactant protein B biosynthesis

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Compound SFTPB 1549C-->GAA (121ins2) and 457delC heterozygosity in severe congenital lung disease and surfactant protein B (SP-B) deficiency

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Structure and properties of surfactant protein B

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Altered surfactant homeostasis and alveolar type II cell morphology in mice lacking surfactant protein D

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

The cloning of a human ABC gene (ABC3) mapping to chromosome 16p13.3

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Primary structure of a novel ABC transporter with a chromosomal localization on the band encoding the multidrug resistance-associated protein

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Developmental expression of SP-A and SP-A mRNA in the proximal and distal respiratory epithelium in the human fetus and newborn

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Intracellular processing of pulmonary surfactant protein B in an endosomal/lysosomal compartment

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ATP-binding cassette (ABC) transporters in human metabolism and diseases

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant lipid synthesis and lamellar body formation in glycogen-laden type II cells

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Identification of LBM180, a lamellar body limiting membrane protein of alveolar type II cells, as the ABC transporter protein ABCA3

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Pulmonary-specific expression of SP-D corrects pulmonary lipid accumulation in SP-D gene-targeted mice

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Processing of surfactant protein B proprotein by a cathepsin D-like protease

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer.

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Pulmonary alveolar proteinosis caused by deletion of the GM-CSFRalpha gene in the X chromosome pseudoautosomal region 1.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Aberrant catalytic cycle and impaired lipid transport into intracellular vesicles in ABCA3 mutants associated with nonfatal pediatric interstitial lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Mutations linked to interstitial lung disease can abrogate anti-amyloid function of prosurfactant protein C.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant collectins and innate immunity

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Structure of pulmonary surfactant membranes and films: the role of proteins and lipid-protein interactions.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant proteins SP-B and SP-C and their precursors in bronchoalveolar lavages from children with acute and chronic inflammatory airway disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Population and disease-based prevalence of the common mutations associated with surfactant deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Diffuse lung disease in young children: application of a novel classification scheme

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Heterozygosity for ABCA3 mutations modifies the severity of lung disease associated with a surfactant protein C gene (SFTPC) mutation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetic disorders of surfactant proteins

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABCA3-mediated choline-phospholipids uptake into intracellular vesicles in A549 cells.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Unexplained neonatal respiratory distress due to congenital surfactant deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Regulation of surfactant secretion in alveolar type II cells.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Pulmonary collectins in innate immunity of the lung

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABC A-subclass proteins: gatekeepers of cellular phospho- and sphingolipid transport.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Novel mutations in the gene encoding ATP binding cassette protein member A3 (ABCA3) resulting in fatal neonatal lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant protein polymorphisms and neonatal lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABCA3 deficiency: neonatal respiratory failure and interstitial lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Inherited surfactant protein-B deficiency and surfactant protein-C associated disease: clinical features and evaluation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetic basis of respiratory distress syndrome

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Defects in surfactant synthesis: clinical implications

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Characterization and classification of ATP-binding cassette transporter ABCA3 mutants in fatal surfactant deficiency.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

A protocol for the handling of tissue obtained by operative lung biopsy: recommendations of the chILD pathology co-operative group

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetics of pediatric interstitial lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surface tension influences cell shape and phagocytosis in alveolar macrophages

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Protein-lipid interactions and surface activity in the pulmonary surfactant system.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

The ABCA subfamily--gene and protein structures, functions and associated hereditary diseases.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

In defense of the lung: surfactant protein A and surfactant protein D.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABC A-subfamily transporters: structure, function and disease.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Functional and trafficking defects in ATP binding cassette A3 mutants associated with respiratory distress syndrome

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Increased and prolonged pulmonary fibrosis in surfactant protein C-deficient mice following intratracheal bleomycin

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABC proteins: key molecules for lipid homeostasis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetic disorders of surfactant homeostasis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABCA3 mutations associated with pediatric interstitial lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant protein C biosynthesis and its emerging role in conformational lung disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Exocytosis of lung surfactant: from the secretory vesicle to the air-liquid interface

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Nonspecific interstitial pneumonia, alveolar proteinosis, and abnormal proprotein trafficking resulting from a spontaneous mutation in the surfactant protein C gene

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Pulmonary alveolar proteinosis in children

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Human ABCA3, a product of a responsible gene for abca3 for fatal surfactant deficiency in newborns, exhibits unique ATP hydrolysis activity and generates intracellular multilamellar vesicles.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Phospholipid metabolism in lung surfactant.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Genetic mechanisms of surfactant deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Host defense functions of pulmonary surfactant

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

N-terminally extended surfactant protein (SP) C isolated from SP-B-deficient children has reduced surface activity and inhibited lipopolysaccharide binding

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABCA3 gene mutations in newborns with fatal surfactant deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Expression of a Human Surfactant Protein C Mutation Associated with Interstitial Lung Disease Disrupts Lung Development in Transgenic Mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Involvement of napsin A in the C- and N-terminal processing of surfactant protein B in type-II pneumocytes of the human lung.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

In vitro surfactant protein B deficiency inhibits lamellar body formation.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Hydrophobic surfactant proteins in lung function and disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Biogenesis of lamellar bodies, lysosome-related organelles involved in storage and secretion of pulmonary surfactant

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Palmitoylation and processing of the lipopeptide surfactant protein C.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

29 September 2017

Biology of surfactant

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Localization and functions of SP-A and SP-D at mucosal surfaces.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Pulmonary collectins and innate host defense of the lung

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Biosynthesis of surfactant protein C: characterization of aggresome formation by EGFP chimeras containing propeptide mutants lacking conserved cysteine residues

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Recurrent familial neonatal deaths: hereditary surfactant protein B deficiency

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Association between the surfactant protein A (SP-A) gene locus and respiratory-distress syndrome in the Finnish population

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Lamellar body membrane turnover is stimulated by secretagogues

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

The role of lipids in pulmonary surfactant.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Generation and characterization of monoclonal antibodies to alveolar type II cell lamellar body membrane

29 September 2017

1 reference

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Measurement of pulmonary status and surfactant protein levels during dexamethasone treatment of neonatal respiratory distress syndrome

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Altered surfactant function and structure in SP-A gene targeted mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Roles of SP-A, SP-B, and SP-C in modulation of lipid uptake by pulmonary epithelial cells in vitro

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Molecular and cellular processing of lung surfactant.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Pathophysiology and treatment of surfactant protein-B deficiency.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Human surfactant protein B: structure, function, regulation, and genetic disease

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Sensitivity and specificity of the L/S ratio in relation to gestational age.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Diagnosis of fetal lung maturity

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Assessment of foetal lung maturity.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Function and regulation of expression of pulmonary surfactant-associated proteins

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant phospholipids: synthesis and storage

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant protein C: a review of its unique properties and metabolism

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Alveolar type II cells: Studies on the mode of release of lamellar bodies

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Macrophage dysfunction and susceptibility to pulmonary Pseudomonas aeruginosa infection in surfactant protein C-deficient mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Regulation and function of pulmonary surfactant protein B.

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant metabolism in surfactant protein A-deficient mice

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Molecular structures and interactions of pulmonary surfactant components

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant Apoprotein A (SP-A) in Tracheal Aspirates of Newborn Infants with RDS

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

ABCA3 is critical for lamellar body biogenesis in vivo.

2 June 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Posttranslational regulation of surfactant protein B expression.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Differences in regulation between nuclear and cytoplasmic Ca2+ in cultured smooth muscle cells

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Cysteine protease activity is required for surfactant protein B processing and lamellar body genesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Surfactant protein B in type II pneumocytes and intra-alveolar surfactant forms of human lungs.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Expression of ABCA3 in developing lung and other tissues.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Surfactant protein D gene polymorphism associated with severe respiratory syncytial virus infection

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Association between surfactant protein A gene locus and severe respiratory syncytial virus infection in infants

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Sequential targeted deficiency of SP-A and -D leads to progressive alveolar lipoproteinosis and emphysema

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

A major deletion in the surfactant protein-B gene causing lethal respiratory distress.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Transient surfactant protein B deficiency in a term infant with severe respiratory failure

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Origin of the prevalentSFTPBindel g.1549C > GAA (121ins2) mutation causing surfactant protein B (SP-B) deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Ultrastructure of lamellar bodies in congenital surfactant deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Nonspecific interstitial pneumonia and usual interstitial pneumonia with mutation in surfactant protein C in familial pulmonary fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

A common mutation in the surfactant protein C gene associated with lung disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Clinical features and genetic analysis of surfactant protein C in adult-onset familial interstitial pneumonia.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Neonatal respiratory failure associated with mutation in the surfactant protein C gene.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Pneumonitis and emphysema in sp-C gene targeted mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

ABCA3 deficiency presenting as persistent pulmonary hypertension of the newborn.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Heterozygous ABCA3 mutation associated with non-fatal evolution of respiratory distress.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling/degeneration (giant lamellar body degeneration) of type-2 pneumocytes.

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

ABCA3 as a Lipid Transporter in Pulmonary Surfactant Biogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Association of polymorphisms in the human surfactant protein-D (SFTPD) gene and postnatal pulmonary adaptation in the preterm infant

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Targeted inactivation of the murine Abca3 gene leads to respiratory failure in newborns with defective lamellar bodies

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Surfactant Composition and Function in Patients with ABCA3 Mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.2350%2F09-01-0586.1

Characteristics of surfactant from SP-A-deficient mice.

21 January 2018

1 reference

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Aberrant processing of surfactant protein C in hereditary SP-B deficiency

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Partial deficiency of surfactant protein B in an infant with chronic lung disease

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2987676

Surfactant protein deficiency in familial interstitial lung disease

27 November 2018

1 reference

12 December 2020

Persistent tachypnea and hypoxia in a 3-month-old term infant

1 reference

12 December 2020

Posttranslational processing of surfactant protein C in rat type II cells

1 reference

12 December 2020

Lung transplantation for treatment of infants with surfactant protein B deficiency

1 reference

12 December 2020

Congenital alveolar proteinosis in the Netherlands: a report of five cases with immunohistochemical and genetic studies on surfactant apoproteins

1 reference

12 December 2020

Pulmonary alveolar proteinosis

1 reference

12 December 2020

Mutation of SFTPC in infantile pulmonary alveolar proteinosis with or without fibrosing lung disease

1 reference

12 December 2020

Progressive lung disease and surfactant dysfunction with a deletion in surfactant protein C gene

1 reference

12 December 2020

Surfactant protein-D regulates surfactant phospholipid homeostasis in vivo

1 reference

12 December 2020

Structure and properties of surfactant protein C

1 reference

12 December 2020

Synthesis, processing and secretion of surfactant proteins B and C

1 reference

12 December 2020

Surfactant protein A (SP-A) gene targeted mice

1 reference

12 December 2020

Genetics of the hydrophobic surfactant proteins

1 reference

12 December 2020

Normal lung function in subjects heterozygous for surfactant protein-B deficiency

1 reference

12 December 2020

Surfactant protein B deficiency: clinical, histological and molecular evaluation

1 reference

12 December 2020

Variable phenotype associated with SP-C gene mutations: fatal case with the I73T mutation

1 reference

12 December 2020

Surfactant protein D influences surfactant ultrastructure and uptake by alveolar type II cells

1 reference

12 December 2020

Absence of lamellar bodies with accumulation of dense bodies characterizes a novel form of congenital surfactant defect

1 reference

12 December 2020

Surfactant metabolism in SP-D gene-targeted mice

1 reference

12 December 2020

Prolonged survival in hereditary surfactant protein B (SP-B) deficiency associated with a novel splicing mutation

1 reference

12 December 2020

Surface-expressed lamellar body membrane is recycled to lamellar bodies

1 reference

12 December 2020

Surfactant protein B deficiency caused by a novel mutation involving multiple exons of the SP-B gene

1 reference

12 December 2020

Usual interstitial pneumonia in an adolescent with ABCA3 mutations

1 reference

12 December 2020

Hereditary surfactant protein B deficiency resulting from a novel mutation

1 reference

12 December 2020

Pulmonary surfactant metabolism in infants lacking surfactant protein B

1 reference

12 December 2020

Population-based estimates of surfactant protein B deficiency

1 reference

12 December 2020

Allelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency

1 reference

12 December 2020

Deficiency of lamellar bodies in alveolar type II cells associated with fatal respiratory disease in a full-term infant

1 reference

12 December 2020

Lamellar body formation in normal and surfactant protein B-deficient fetal mice

1 reference

12 December 2020

Hermansky-Pudlak syndrome and Chediak-Higashi syndrome: disorders of vesicle formation and trafficking

1 reference

12 December 2020

Population-based screening for rare mutations: high-throughput DNA extraction and molecular amplification from Guthrie cards

1 reference

12 December 2020

Mutations in the surfactant protein C gene associated with interstitial lung disease

1 reference

12 December 2020

Immunolocalization of surfactant protein-D (SP-D) in human fetal, newborn, and adult tissues

1 reference

12 December 2020

Pulmonary alveolar proteinosis

1 reference

12 December 2020

Alteration of the Pulmonary Surfactant System in Full-Term Infants with Hereditary ABCA3 Deficiency

1 reference

12 December 2020

Increased synthesis and mRNA of surfactant protein A in oxygen-exposed rats

1 reference

12 December 2020

Surfactant protein-A in bronchoalveolar lavage fluid from neonates with RDS on conventional and high-frequency oscillatory ventilation

1 reference

12 December 2020

The ontogeny and distribution of surfactant protein B in human fetuses and newborns

1 reference

12 December 2020

Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis

1 reference

12 December 2020

Surfactant protein B deficiency: radiographic manifestations

1 reference

12 December 2020

Biosynthetic routing of pulmonary surfactant proteins in alveolar type II cells

1 reference

12 December 2020

Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency

1 reference

12 December 2020

Ultrastructure of lung in surfactant protein B deficiency

1 reference

12 December 2020

Localization, synthesis, and processing of surfactant protein SP-C in rat lung analyzed by epitope-specific antipeptide antibodies

1 reference

12 December 2020

Temporal-spatial distribution of SP-B and SP-C proteins and mRNAs in developing respiratory epithelium of human lung

1 reference

12 December 2020

Surfactant protein B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement

1 reference

12 December 2020

Analysis of 40 sporadic or familial neonatal and pediatric cases with severe unexplained respiratory distress: relationship to SFTPB

1 reference

12 December 2020

Surfactant protein C mutations in sporadic forms of idiopathic interstitial pneumonias

1 reference

12 December 2020

ABCA3 inactivation in mice causes respiratory failure, loss of pulmonary surfactant, and depletion of lung phosphatidylglycerol

1 reference

12 December 2020

Ultrastructural and molecular analysis in fatal neonatal interstitial pneumonia caused by a novel ABCA3 mutation

1 reference

12 December 2020

Clinical, radiological and pathological features of ABCA3 mutations in children

1 reference

12 December 2020