Surfactant protein C is part of the surfactant complex lining up the alveoles and thereby
inhibiting collapse of the airways. In addition it is involved in innate immune responses. Rare …

В обзоре на основании данных современной литературы представлены сведения о
биологии, физиологии и метаболизме сурфактанта, идентифицированных в …

Publisher Summary This chapter focuses on the normal composition, function, and
development of the pulmonary surfactant system, and discusses the effects of genetic and …

Familial interstitial lung disease (ILD) is defined as presence of ILD in 2 or more family
members. Surfactant protein C (SFTPC) gene mutations are rare, but well‐known cause of …

Genetic mechanisms are now recognized as rare causes of neonatal lung disease. Genes
potentially responsible for neonatal lung disease include those encoding proteins important …

Pulmonary surfactant is a highly surface-active mixture of proteins and lipids that is
synthesized and secreted in the alveoli by type II epithelial cells and is found in the fluid …

Idiopathic pulmonary fibrosis (IPF) is a severe interstitial lung disease; although the recent
introduction of two anti-fibrosis drugs, pirfenidone and Nidanib, have resulted in a significant …

Background Heterozygous mutations of SFTPC, the gene encoding surfactant protein C (SP-
C), cause sporadic and familial interstitial lung disease (ILD) in children and adults. The …

A multi‐disciplinary scientific conference focused on diffuse and interstitial lung diseases in
children was held in La Jolla, CA in June 2012. The conference brought together clinicians …