Structural requirements for intracellular transport of pulmonary surfactant protein B (SP-B)
نویسندگان
چکیده
منابع مشابه
Allelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency.
Inability to produce surfactant protein B (SP-B) causes fatal neonatal respiratory disease. A frame-shift mutation (121ins2) is the predominant but not exclusive cause of disease. To determine the range of mechanisms responsible for SP-B deficiency, both alleles from 32 affected infants were characterized. Sixteen infants were homozygous for the 121ins2 mutation, 10 infants were heterozygous fo...
متن کاملSP-B refining of pulmonary surfactant phospholipid films.
Pulmonary surfactant stabilizes the alveoli by lining the air-fluid interface with films that reduce surface tension to near 0 mN/m (γmin). Surfactant protein B (SP-B) enhances the surface activity of surfactant phospholipids. A captive bubble tensiometer (CBT) was used to study the properties of adsorbed films of dipalmitoylphosphatidylcholine (DPPC) with acidic 1-palmitoyl-2-oleoyl- sn-glycer...
متن کاملPulmonary surfactant metabolism in infants lacking surfactant protein B.
Infants with inherited deficiency of pulmonary surfactant protein (SP) B develop respiratory failure at birth and die without lung transplantation. We examined aspects of surfactant metabolism in lung tissue and lavage fluid acquired at transplantation or postmortem from ten infants born at term with inherited deficiency of SP-B; comparison groups were infants with other forms of chronic lung d...
متن کاملSurfactant protein B: unambiguously necessary for adult pulmonary function.
IN THE REPORT FROM MELTON ET AL., one of the current articles in focus (Ref. 21, see p. L543 in this issue), they use genetically engineered mice to demonstrate that at least 25% of normal surfactant protein B production is required for adult pulmonary function. With the use of doxycycline-regulated, compound-conditional knockout murine lineages, the authors show that genetic disruption of surf...
متن کاملEffect of pulmonary surfactant protein SP-B on the micro- and nanostructure of phospholipid films.
Monolayers of dipalmitoylphosphatidylcholine (DPPC) and DPPC/dipalmitoylphosphatidylglycerol (DPPG) (7:3, w/w) in the absence or in the presence of 2, 5, 10, or 20 weight percent of porcine surfactant protein SP-B were spread at the air-liquid interface of a surface balance, compressed up to surface pressures in the liquid-expanded/liquid-condensed (LE-LC) plateau of the isotherm, transferred o...
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ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
سال: 1996
ISSN: 0167-4889
DOI: 10.1016/0167-4889(95)00201-4