1997
DOI: 10.1074/jbc.272.15.9640
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Rescue of SP-B Knockout Mice with a Truncated SP-B Proprotein

Abstract: Pulmonary surfactant is a complex mixture of lipids and proteins that is synthesized exclusively by the alveolar type II epithelial cell. Surfactant is stored in large inclusions (lamellar bodies) that are secreted into the alveolar airspace by exocytosis. Newly secreted lamellar bodies unravel into a tubular network (tubular myelin) that subsequently adsorbs and spreads as a phospholipid-rich film that reduces surface tension at the air-liquid interface. Rapid adsorption and spreading of the phospholipid film… Show more

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Cited by 69 publications
(54 citation statements)
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“…SP-B, a dimer of 16 kDa, was detected easily in all cases investigated except for the single case of hereditary SP-B deficiency. Together with the finding of lacking SP-B in all other reported cases of this genetic disorder (7,17,18,(24)(25)(26) and the successful rescue of SP-B-deficient knockout mice (27), the analysis of BALF for SP-B concentration appears diagnostically helpful when pediatric DPLD potentially including this rare condition are investigated. In this context, it must be recommended to sample BAL before exogenous surfactant is applied, as the latter usually contains both SP-B and SP-C whose in vivo half-lives may be very long (28)(29)(30).…”
Section: Discussionmentioning
confidence: 99%
“…SP-B, a dimer of 16 kDa, was detected easily in all cases investigated except for the single case of hereditary SP-B deficiency. Together with the finding of lacking SP-B in all other reported cases of this genetic disorder (7,17,18,(24)(25)(26) and the successful rescue of SP-B-deficient knockout mice (27), the analysis of BALF for SP-B concentration appears diagnostically helpful when pediatric DPLD potentially including this rare condition are investigated. In this context, it must be recommended to sample BAL before exogenous surfactant is applied, as the latter usually contains both SP-B and SP-C whose in vivo half-lives may be very long (28)(29)(30).…”
Section: Discussionmentioning
confidence: 99%
“…Mature SP-B corresponds to the middle (34,35). Genetically engineered mice that expressed only SP-B constructs that lacked sequences encoding the proSP-B carboxy terminal domains produced mature SP-B but had perturbed proSP-C processing and abnormally large LB, suggesting a role for the carboxy terminal region in LB biosynthesis and SP-C proprotein processing (36). However, SP-C metabolism was abnormal in the children we describe despite the presence of the aberrant protein, suggesting that the carboxy terminal region of the abnormal peptide was nonfunctional or that the reduced level of mature SP-B protein was also important in altered proSP-C processing.…”
Section: Prolonged Survival In Sp-b Deficiencymentioning
confidence: 99%
“…Supernatants were then analyzed for the four surfactant proteins, SP-A, SP-B, SP-C, and SP-D. Lavage content of SP-B and SP-D was measured by specific enzyme-linked immunosorbent assay (ELISA) as previously reported. [22][23][24] Western blots were performed for SP-A and the mature form of SP-C using polyclonal antibodies as previously described. 17 Densitometry was performed using Gelworks 1D Advanced V3.01 software (Ultra-Violet Products, Cambridge, UK) and the individual band density was normalized to a positive control run on each gel.…”
Section: Analysis Of Surfactant Proteins In Lung Lavagementioning
confidence: 99%