Disease: Pulmonary surfactant protein B- deficiency of
- <em>ABCA3</em> deficiency from birth to adulthood presenting as paediatric interstitial lung disease
- A dominant negative variant of <em>RAB5B</em> disrupts maturation of surfactant protein B and surfactant protein C
- A dominant negative variant of RAB5B disrupts maturation of surfactant protein B and surfactant protein C
- A lung tropic AAV vector improves survival in a mouse model of surfactant B deficiency
- A model for the structure and mechanism of action of pulmonary surfactant protein B
- A New <em>ABCA3</em> Gene Mutation c.3445G>A (p.Asp1149Asn) as a Causative Agent of Newborn Lethal Respiratory Distress Syndrome
- A novel surgical approach for intratracheal administration of bioactive agents in a fetal mouse model
- A promoterless AAV6.2FF-based lung gene editing platform for the correction of surfactant protein B deficiency
- A rare large mutation involving two exons of the SP-B gene in an infant with severe respiratory distress
- ABCG1 regulates pulmonary surfactant metabolism in mice and men
- Activation of the GLP-1 Receptor by Liraglutide Increases ACE2 Expression, Reversing Right Ventricle Hypertrophy, and Improving the Production of SP-A and SP-B in the Lungs of Type 1 Diabetes Rats
- Activity and biophysical inhibition resistance of a novel synthetic lung surfactant containing Super-Mini-B DATK peptide
- Aerosol delivery of dry powder synthetic lung surfactant to surfactant-deficient rabbits and preterm lambs on non-invasive respiratory support
- Aggravated pulmonary injury after subarachnoid hemorrhage in PDGF-B<sup>ret/ret</sup> mice
- BMP signaling is essential in neonatal surfactant production during respiratory adaptation
- Changes in respiratory elastance after deep inspirations reflect surface film functionality in mice with acute lung injury
- Congenital Surfactant C Deficiency with Pulmonary Hypertension-A Case Report
- Congenital surfactant protein B (SP-B) deficiency: a case report
- Deficiency in pulmonary surfactant proteins in mice with fatty acid binding protein 4-Cre-mediated knockout of the tuberous sclerosis complex 1 gene
- Deficiency of surfactant protein: Case report
- Delayed Presentation and Prolonged Survival of a Child with Surfactant Protein B Deficiency
- Deletion of STK40 protein in mice causes respiratory failure and death at birth
- Development of Aging-Related Emphysematous and Lymphoma-Like Lesions is Enhanced by the Lack of Secretoglobin 3A2 in Mouse Lungs
- Diagnosis of asphyxiation in advanced decomposed corpses by using Oil-Red-O Stain and immunohistochemical technology
- Difficulties in the treatment of an infant survivor with inherited surfactant protein-B deficiency in Tunisia
- Evidence reviews for respiratory support: Specialist neonatal respiratory care for babies born preterm: Evidence review B
- Evolution of surfactant therapy for respiratory distress syndrome: past, present, and future
- Exogenous gene transfer of Rab(3)8 small GTPase ameliorates aberrant lung surfactant homeostasis in Ruby rats
- Exogenous gene transfer of Rab<sup>3</sup>8 small GTPase ameliorates aberrant lung surfactant homeostasis in Ruby rats
- Expression and potential regulation of miRNA‑431 during lung development of Sprague‑Dawley rats
- Featured Article: Electroporation-mediated gene delivery of surfactant protein B (SP-B) restores expression and improves survival in mouse model of SP-B deficiency
- Fgf10 deficiency is causative for lethality in a mouse model of bronchopulmonary dysplasia
- GATA2 Related Conditions and Predisposition to Pediatric Myelodysplastic Syndromes
- Gene Therapy Potential for Genetic Disorders of Surfactant Dysfunction
- Genetic basis of surfactant dysfunction in Chinese children: A retrospective study
- Genetic susceptibility to neonatal lung diseases
- Genotype alone does not predict the clinical course of SFTPC deficiency in paediatric patients
- Gestational vitamin D deficiency impairs fetal lung development through suppressing type II pneumocyte differentiation
- Hereditary interstitial lung diseases manifesting in early childhood in Japan
- Heterogeneity of lung disease associated with NK2 homeobox 1 mutations
- Identification of vitamin D sensitive pathways during lung development
- IgA modulates respiratory dysfunction as a sequela to pulmonary chlamydial infection as neonates
- Immunophenotyping of Acute Inflammatory Exacerbations of Lung Injury Driven by Mutant Surfactant Protein-C: A Role for Inflammatory Eosinophils
- In vitro and in vivo comparison between poractant alfa and the new generation synthetic surfactant CHF5633
- In vivo genome editing using nuclease-encoding mRNA corrects SP-B deficiency
- Late administration of surfactant replacement therapy increases surfactant protein-B content: a randomized pilot study
- Lethal influenza infection: Is a macrophage to blame?
- Lkb1 loss promotes tumor progression of BRAF(V600E)-induced lung adenomas
- Lucinactant for the prevention of respiratory distress syndrome in premature infants
- Lung Surfactant Protein B Peptide Mimics Interact with the Human ACE2 Receptor
- Maternal bile acid transporter deficiency promotes neonatal demise
- MCRIP1 promotes the expression of lung-surfactant proteins in mice by disrupting CtBP-mediated epigenetic gene silencing
- Natural Anti-Infective Pulmonary Proteins: In Vivo Cooperative Action of Surfactant Protein SP-A and the Lung Antimicrobial Peptide SP-BN
- New Directions in Pulmonary Gene Therapy
- New surfactant with SP-B and C analogs gives survival benefit after inactivation in preterm lambs
- Null ABCA3 in humans: large homozygous ABCA3 deletion, correlation to clinical-pathological findings
- Outcomes of Lung Transplantation for Infants and Children with Genetic Disorders of Surfactant Metabolism
- p120-catenin expressed in alveolar type II cells is essential for the regulation of lung innate immune response
- Persistent Respiratory Distress in the Term Neonate: Genetic Surfactant Deficiency Diseases
- Phycocyanin attenuates pulmonary fibrosis via the TLR2-MyD88-NF-κB signaling pathway
- Prenatal administration of all-trans retinoic acid upregulates leptin signaling in hypoplastic rat lungs with experimental congenital diaphragmatic hernia
- Pulmonary glycogen deficiency as a new potential cause of respiratory distress syndrome
- Pulmonary surfactant preserves viability of alveolar type II cells exposed to polymyxin B in vitro
- Quenching of tryptophan fluorescence in a highly scattering solution: Insights on protein localization in a lung surfactant formulation
- Respiratory distress and early neonatal lethality in Hspa4l/Hspa4 double-mutant mice
- Reversal of Surfactant Protein B Deficiency in Patient Specific Human Induced Pluripotent Stem Cell Derived Lung Organoids by Gene Therapy
- RNA-seq analysis of the human surfactant air-liquid interface culture reveals alveolar type II cell-like transcriptome
- Secretoglobin 3A2 Exhibits Anti-Fibrotic Activity in Bleomycin-Induced Pulmonary Fibrosis Model Mice
- Sigmar1 ablation leads to lung pathological changes associated with pulmonary fibrosis, inflammation, and altered surfactant proteins levels
- SP-A and SP-D: Dual Functioning Immune Molecules With Antiviral and Immunomodulatory Properties
- SP-B and SP-C containing new synthetic surfactant for treatment of extremely immature lamb lung
- Stem cells for neonatal lung disease caused by SP-B deficiency?
- Streptococcus pneumoniae triggers progression of pulmonary fibrosis through pneumolysin
- Structural and functional stability of the sulfur-free surfactant protein B peptide mimic B-YL in synthetic surfactant lipids
- Structural and transcriptomic response to antenatal corticosteroids in an Erk3-null mouse model of respiratory distress
- Study of novel artificial lung surfactants incorporating partially fluorinated amphiphiles
- Suppression of pulmonary group B streptococcal proliferation and translocation by surfactants in ventilated near-term newborn rabbits
- Surface film formation in vitro by infant and therapeutic surfactants: role of surfactant protein B
- Surfactant dysfunction and lung inflammation in the female mouse model of lymphangioleiomyomatosis
- Surfactant Protein A, a Novel Regulator for Smooth Muscle Phenotypic Modulation and Vascular Remodeling-Brief Report
- Surfactant protein B deficiency and gene mutations for neonatal respiratory distress syndrome in China Han ethnic population
- Surfactant Protein B Deficiency Induced High Surface Tension: Relationship between Alveolar Micromechanics, Alveolar Fluid Properties and Alveolar Epithelial Cell Injury
- Surfactant protein B: From biochemistry to its potential role as diagnostic and prognostic marker in heart failure
- Surfactant protein disorders in childhood interstitial lung disease
- Surfactant proteins in pediatric interstitial lung disease
- Surfactant status and respiratory outcome in premature infants receiving late surfactant treatment
- Surfactant-associated proteins: structure, function and clinical implications
- Synthetic lung surfactant reduces alveolar-capillary protein leakage in surfactant-deficient rabbits
- Synthetic lung surfactants containing SP-B and SP-C peptides plus novel phospholipase-resistant lipids or glycerophospholipids
- Targeted Disruption of Ig-Hepta/Gpr116 Causes Emphysema-like Symptoms That Are Associated with Alveolar Macrophage Activation
- The Common Haplotype GATGACA in Surfactant-Protein B Gene Is Protective for Respiratory Distress Syndrome in Preterm Neonates
- The effect of matrix metalloproteinase-3 deficiency on pulmonary surfactant in a mouse model of acute lung injury
- The role of carnitine in the perinatal period
- The role of miR-431-5p in regulating pulmonary surfactant expression in vitro
- The Role of Surfactant in Lung Disease and Host Defense against Pulmonary Infections
- Towards personalized therapies for genetic disorders of surfactant dysfunction
- Transcription repressor Bach2 is required for pulmonary surfactant homeostasis and alveolar macrophage function
- Ultrastructural characterization of genetic diffuse lung diseases in infants and children: a cohort study and review
- Up-regulation of SFTPB expression and attenuation of acute lung injury by pulmonary epithelial cell-specific NAMPT knockdown
- Use of Berlin EXCOR cannulas in both venovenous and venoarterial central extracorporeal membrane oxygenation configurations overcomes the problem of cannula instability while bridging infants and young children to lung transplant