Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse

Air Space Distension Precedes Spontaneous Fibrotic Remodeling and Impaired Cholesterol Metabolism in the Absence of Surfactant Protein C

(2020) Jannik Ruwisch et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

A systematic approach to quantitative Western blot analysis

(2020) Lakshmi Pillai-Kastoori et al.   ANALYTICAL BIOCHEMISTRY

Brain Pathology in Mucopolysaccharidoses (MPS) Patients with Neurological Forms

(2020) Gustavo M. Viana et al.   Journal of Clinical Medicine

On Top of the Alveolar Epithelium: Surfactant and the Glycocalyx

(2020) Matthias Ochs et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Gaucher disease type 3c: New patients with unique presentations and review of the literature

(2019) Alina Kurolap et al.   MOLECULAR GENETICS AND METABOLISM

Synthetic Disaccharide Standards Enable Quantitative Analysis of Stored Heparan Sulfate in MPS IIIA Murine Brain Regions

(2019) Qi Qi He et al.   ACS Chemical Neuroscience

Intracellular cholesterol trafficking is dependent upon NPC2 interaction with lysobisphosphatidic acid

(2019) Leslie A McCauliff et al.   eLife

Epithelial Heparan Sulfate Contributes to Alveolar Barrier Function and is Shed During Lung Injury

(2018) Sarah M Haeger et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Pulmonary surfactant protein SP-B promotes exocytosis of lamellar bodies in alveolar type II cells

(2018) Marta Martínez-Calle et al.   FASEB JOURNAL

Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis

(2018) Aleksandra Somogyi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Maternal undernutrition in late gestation increases IGF2 signalling molecules and collagen deposition in the right ventricle of the fetal sheep heart

(2018) Jack R. T. Darby et al.   JOURNAL OF PHYSIOLOGY-LONDON

Understanding the principle biophysics concepts of pulmonary surfactant in health and disease

(2018) Chiara Autilio et al.   Archives of Disease in Childhood-Fetal and Neonatal Edition

Mucopolysaccharidosis type IIIA storage substrate drives an innate immune neuro-inflammatory response

(2017) Brian Bigger et al.   MOLECULAR GENETICS AND METABOLISM

Evaluation of pentosan polysulfate in mucopolysaccharidosis type IIIA mice

(2017) Calogera M. Simonaro et al.   MOLECULAR GENETICS AND METABOLISM

Mortality in patients with Sanfilippo syndrome

(2017) Christine Lavery et al.   Orphanet Journal of Rare Diseases

Lipidomics reveals dramatic lipid compositional changes in the maturing postnatal lung

(2017) Sydney E. Dautel et al.   Scientific Reports

Bronchoalveolar Lavage of Murine Lungs to Analyze Inflammatory Cell Infiltration

(2017) Lien Van Hoecke et al.   Jove-Journal of Visualized Experiments

Heparan Sulfate in the Developing, Healthy, and Injured Lung

(2016) Sarah M. Haeger et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Lung remodeling associated with recovery from acute lung injury

(2016) Sean E. Gill et al.   CELL AND TISSUE RESEARCH

The role of cholesterol in membrane fusion

(2016) Sung-Tae Yang et al.   CHEMISTRY AND PHYSICS OF LIPIDS

Glycosaminoglycan levels and structure in a mucopolysaccharidosis IIIA mice and the effect of a highly secreted sulfamidase engineered to cross the blood-brain barrier

(2016) F Maccari et al.   METABOLIC BRAIN DISEASE

Composition, structure and mechanical properties define performance of pulmonary surfactant membranes and films

(2015) Elisa Parra et al.   CHEMISTRY AND PHYSICS OF LIPIDS

Surfactant proteins in pediatric interstitial lung disease

(2015) Matthias Griese et al.   PEDIATRIC RESEARCH

Overcoming inactivation of the lung surfactant by serum proteins: a potential role for fluorocarbons?

(2015) Marie Pierre Krafft   Soft Matter

Structure-function relationships in pulmonary surfactant membranes: From biophysics to therapy

(2014) Elena Lopez-Rodriguez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Macrophage Dysfunction and Susceptibility to Pulmonary Pseudomonas aeruginosa Infection in Surfactant Protein C-Deficient Mice

(2014) S. W. Glasser et al.   JOURNAL OF IMMUNOLOGY

Surface film formation in vitro by infant and therapeutic surfactants: role of surfactant protein B

(2014) Olivier Danhaive et al.   PEDIATRIC RESEARCH

Tachypnea of Infancy as the First Sign of Sanfilippo Syndrome

(2014) J. Chiang et al.   PEDIATRICS

Mucopolysaccharide diseases: A complex interplay between neuroinflammation, microglial activation and adaptive immunity

(2013) Louise D. Archer et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Pathophysiology of heparan sulphate: many diseases, few drugs

(2013) U. Lindahl et al.   JOURNAL OF INTERNAL MEDICINE

Characterization of the Niemann-Pick C pathway in alveolar type II cells and lamellar bodies of the lung

(2012) Blair R. Roszell et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Regulation of lung surfactant phospholipid synthesis and metabolism

(2012) Victoria Goss et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Ganglioside GM3 Has an Essential Role in the Pathogenesis and Progression of Rheumatoid Arthritis

(2012) Yukinori Tsukuda et al.   PLoS One

Maternal undernutrition reduces P-glycoprotein in guinea pig placenta and developing brain in late gestation

(2012) Poh S. Soo et al.   REPRODUCTIVE TOXICOLOGY

Bacterial Lipopolysaccharide Promotes Destabilization of Lung Surfactant-Like Films

(2011) Olga Cañadas et al.   BIOPHYSICAL JOURNAL

Analysis of lung surfactant phosphatidylcholine metabolism in transgenic mice using stable isotopes

(2011) Anthony D. Postle et al.   CHEMISTRY AND PHYSICS OF LIPIDS

The cellular pathology of lysosomal diseases

(2011) Timothy M Cox et al.   JOURNAL OF PATHOLOGY

Cognitive development in patients with Mucopolysaccharidosis type III (Sanfilippo syndrome)

(2011) Marlies J Valstar et al.   Orphanet Journal of Rare Diseases

Heparan Sulfates in the Lung: Structure, Diversity, and Role in Pulmonary Emphysema

(2010) Nicole C. Smits et al.   Anatomical Record-Advances in Integrative Anatomy and Evolutionary Biology

Palmitoylation of Pulmonary Surfactant Protein SP-C Is Critical for Its Functional Cooperation with SP-B to Sustain Compression/Expansion Dynamics in Cholesterol-Containing Surfactant Films

(2010) Florian Baumgart et al.   BIOPHYSICAL JOURNAL

Biological Function of the Cellular Lipid BMP—BMP as a Key Activator for Cholesterol Sorting and Membrane Digestion

(2010) Hichem D. Gallala et al.   NEUROCHEMICAL RESEARCH

Respiratory Manifestations in Mucopolysaccharidoses

(2010) Marianne S. Muhlebach et al.   Paediatric Respiratory Reviews

Pulmonary Surfactant Pathophysiology: Current Models and Open Questions

(2010) Jesús Perez-Gil et al.   PHYSIOLOGY

Role of cholesterol in the biophysical dysfunction of surfactant in ventilator-induced lung injury

(2009) Dan Vockeroth et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Bis(monoacylglycero)phosphate Forms Stable Small Lamellar Vesicle Structures: Insights into Vesicular Body Formation in Endosomes

(2009) Thomas E. Frederick et al.   BIOPHYSICAL JOURNAL

The MIQE Guidelines: Minimum Information for Publication of Quantitative Real-Time PCR Experiments

(2009) S. A. Bustin et al.   CLINICAL CHEMISTRY

Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis

(2009) M Griese et al.   CLINICAL GENETICS

Surfactant protein-D regulates the postnatal maturation of pulmonary surfactant lipid pool sizes

(2009) Machiko Ikegami et al.   JOURNAL OF APPLIED PHYSIOLOGY

Bis(monoacylglycero)phosphate, a peculiar phospholipid to control the fate of cholesterol: Implications in pathology

(2009) F. Hullin-Matsuda et al.   PROSTAGLANDINS LEUKOTRIENES AND ESSENTIAL FATTY ACIDS

Prominent increase in plasma ganglioside GM3 is associated with clinical manifestations of type I Gaucher disease

(2008) Karen Ghauharali-van der Vlugt et al.   CLINICA CHIMICA ACTA

Sanfilippo syndrome: A mini-review

(2008) M. J. Valstar et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis

(2008) Sabrina Jabs et al.   JOURNAL OF NEUROCHEMISTRY

Type I Gaucher Disease, a Glycosphingolipid Storage Disorder, Is Associated with Insulin Resistance

(2007) Mirjam Langeveld et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM