Background: Genetic and developmental disruption of surfactant protein B (SP-B) expression causes neonatal respiratory distress syndrome (RDS). Objectives: To assess developmental and genetic regulation of SP-B expression in vivo. Methods: To evaluate in vivo developmental regulation of SP-B, we used immunoblotting to compare frequency of detection of mature and pro-SP-B peptides in developmentally distinct cohorts: 24 amniotic fluid samples, unfractionated tracheal aspirates from 101 infants ≥34 weeks’ gestation with (75) and without (26) neonatal RDS, and 6 nonsmoking adults. To examine genetic regulation, we used univariate and logistic regression analyses to detect associations between common SP-B (SFTPB) genotypes and SP-B peptides in the neonatal RDS cohort. Results: We found pro-SP-B peptides in 24/24 amniotic fluid samples and in 100/101 tracheal aspirates from newborn infants but none in bronchoalveolar lavage from normal adults (0/6) (p < 0.001). We detected an association (p = 0.0011) between pro-SP-B peptides (Mr 40 and 42 kDa) and genotype of a nonsynonymous single nucleotide polymorphism at genomic position 1580 that regulates amino-terminus glycosylation. Conclusions: Pro-SP-B peptides are more common in developmentally less mature humans. Association of genotype at genomic position 1580 with pro-SP-B peptides (Mr 40 and 42 kDa) suggests genetic regulation of amino terminus glycosylation in vivo.
© 2008 S. Karger AG, Basel
- Neonatal respiratory diseases
- Respiratory distress syndrome
- Surfactant proteins
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Aaron Hamvas, MD
Division of Newborn Medicine, St. Louis Children’s Hospital
One Children’s Place
St. Louis, MO 63110 (USA)
Tel. +1 314 454 6148, Fax +1 314 454 4633, E-Mail firstname.lastname@example.org
These data were presented at the Pediatric Academic Societies’ Meetings in San Francisco, May 2004 and 2006, and in Washington, D.C., May 2005 (E-PAS 2006:59:2610.4; Pediatr Res 2005;57:2502, and Pediatr Res 2004;55:2664).
Received: December 6, 2007
Accepted: January 21, 2008
Published online: September 6, 2008
Number of Print Pages : 8
Number of Figures : 3, Number of Tables : 5, Number of References : 31
Neonatology (Fetal and Neonatal Research)
Vol. 95, No. 2, Year 2009 (Cover Date: February 2009)
Journal Editor: Halliday H.L. (Belfast), Speer C.P. (Würzburg)
ISSN: 1661–7800 (Print), eISSN: 1661–7819 (Online)
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