Document Detail


Pulmonary surfactant metabolism in infants lacking surfactant protein B.
MedLine Citation:
PMID:  10696076     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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 disease (CLD) and normal infants. In pulse/chase labeling studies with cultured deficient tissue, no immunoprecipitable SP-B was observed and an approximately 6-kD form of SP-C accumulated that was only transiently present in CLD tissue. SP-B messenger RNA (mRNA) was approximately 8% of normal in deficient specimens, and some intact message was observed after, but not before, explant culture. Transcription rates for SP-B, assessed by nuclear run-on assay using probes for sequences both 5' and 3' of the common nonsense mutation (121ins2), were comparable in all lungs examined. The minimal surface tension achieved with lavage surfactant was similarly elevated in both deficient and CLD infants (26-31 mN/m) compared with normal infants (6 mN/m). Both SP-B-deficient and CLD infants had markedly decreased phosphatidylglycerol content of lavage and tissue compared with normal lung, whereas synthetic rates for phospholipids, including phosphatidylglycerol, were normal. We conclude that the mutated SP-B gene is transcribed normally but produces an unstable mRNA and that absence of SP-B protein blocks processing of SP-C. Chronic infant lung disease, of various etiologies, reduces surfactant function and apparently alters phosphatidylglycerol degradation.
Authors:
M F Beers; A Hamvas; M A Moxley; L W Gonzales; S H Guttentag; K O Solarin; W J Longmore; L M Nogee; P L Ballard
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  American journal of respiratory cell and molecular biology     Volume:  22     ISSN:  1044-1549     ISO Abbreviation:  Am. J. Respir. Cell Mol. Biol.     Publication Date:  2000 Mar 
Date Detail:
Created Date:  2000-04-14     Completed Date:  2000-04-14     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  8917225     Medline TA:  Am J Respir Cell Mol Biol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  380-91     Citation Subset:  IM    
Affiliation:
Department of Medicine, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania, and the Institute for Environmental Medicine, USA.
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MeSH Terms
Descriptor/Qualifier:
Acetates / metabolism,  pharmacology
Blotting, Western
Cysteine / pharmacokinetics
Fetus / metabolism
Gene Expression / physiology
Genotype
Humans
Infant
Infant, Newborn
Methionine / pharmacokinetics
Phosphatidylcholines / metabolism
Phosphatidylglycerols / metabolism
Proteolipids / analysis,  genetics*,  metabolism*
Pulmonary Surfactant-Associated Proteins
Pulmonary Surfactants / analysis,  genetics*,  metabolism*
RNA, Messenger / analysis
Respiratory Distress Syndrome, Newborn / metabolism*
Sulfur Radioisotopes / diagnostic use
Transcription, Genetic / physiology
Tritium / diagnostic use
Grant Support
ID/Acronym/Agency:
1P50HL56401/HL/NHLBI NIH HHS; HD54187/HD/NICHD NIH HHS; HL54158/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Acetates; 0/Phosphatidylcholines; 0/Phosphatidylglycerols; 0/Proteolipids; 0/Pulmonary Surfactant-Associated Proteins; 0/Pulmonary Surfactants; 0/RNA, Messenger; 0/Sulfur Radioisotopes; 10028-17-8/Tritium; 52-90-4/Cysteine; 63-68-3/Methionine

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