[HTML][HTML] Gene therapy potential for genetic disorders of surfactant dysfunction

AL Cooney, JA Wambach, PL Sinn… - Frontiers in genome …, 2022 - frontiersin.org
Pulmonary surfactant is critically important to prevent atelectasis by lowering the surface
tension of the alveolar lining liquid. While respiratory distress syndrome (RDS) is common in …

[HTML][HTML] Endogenous human proteins interfering with amyloid formation

AL Gharibyan, S Wasana Jayaweera, M Lehmann… - Biomolecules, 2022 - mdpi.com
Amyloid formation is a pathological process associated with a wide range of degenerative
disorders, including Alzheimer's disease, Parkinson's disease, and diabetes mellitus type 2 …

[HTML][HTML] Abilities of the BRICHOS domain to prevent neurotoxicity and fibril formation are dependent on a highly conserved Asp residue

G Chen, Y Andrade-Talavera, X Zhong… - RSC chemical …, 2022 - pubs.rsc.org
Proteins can self-assemble into amyloid fibrils or amorphous aggregates and thereby cause
disease. Molecular chaperones can prevent both these types of protein aggregation, but to …

Gene therapeutics for surfactant dysfunction disorders: targeting the alveolar type 2 epithelial cell

S Sitaraman, KD Alysandratos, JA Wambach… - Human Gene …, 2022 - liebertpub.com
Genetic disorders of surfactant dysfunction result in significant morbidity and mortality,
among infants, children, and adults. Available medical interventions are limited, nonspecific …

Novel insights into surfactant protein C trafficking revealed through the study of a pathogenic mutant

JA Dickens, EN Rutherford, S Abreu… - European …, 2022 - Eur Respiratory Soc
Background Alveolar epithelial cell dysfunction plays an important role in the pathogenesis
of idiopathic pulmonary fibrosis (IPF), but remains incompletely understood. Some …

[HTML][HTML] Dimerization of the pulmonary surfactant protein C in a membrane environment

H Korolainen, F Lolicato, G Enkavi, J Pérez-Gil… - Plos one, 2022 - journals.plos.org
Surfactant protein C (SP-C) has several functions in pulmonary surfactant. These include the
transfer of lipids between different membrane structures, a role in surfactant recycling and …

[HTML][HTML] Etiologic Profile of older children with diffuse radiological changes in Eastern China

X Xu, L Liu, X Xu, Q Ma, L Teng, H Zhou, L Yang… - Frontiers in …, 2022 - frontiersin.org
Objective: To analyze the etiology of chest diffuse radiological changes (DRC) in children
older than 2 years. Methods A retrospective study was conducted on a primary cohort of …

The Role of ATP Binding Cassette A3 (ABCA3) in Health and Disease Using Pluripotent Stem Cell-Derived Type II Alveolar Epithelial Cells

YL Sun - 2022 - search.proquest.com
The most common causes of childhood interstitial lung disease (chILD) are autosomal
recessive mutations in the gene encoding ATP Binding Cassette A3 (ABCA3) protein, a …