An overview of pulmonary surfactant in the neonate: Genetics, metabolism, and the role of surfactant in health and disease

Nkadi, Paul; Merritt, T. Allen; Pillers, De-Ann M.

doi:10.1016/j.ymgme.2009.01.015

Cited by 164 publications

(132 citation statements)

References 73 publications

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“…RDS usually develops in the first 24 h after the birth of premature babies with developmental insufficiency of pulmonary surfactant synthesis and structurally immature lungs (Clements and Avery, 1998;Gower and Nogee, 2011;Rodriguez et al, 2002;Yurdakok, 2004). Genetic abnormalities of producing surfactant proteins (Spa, Spb, Spc, and/or Spd) can result in this syndrome (Beers et al, 2000;Gower and Nogee, 2011;Nkadi et al, 2009;Nogee, 2004).…”

Section: Introductionmentioning

confidence: 99%

Pbx1 activates Fgf10 in the mesenchyme of developing lungs

Lin

Shang

et al. 2014

Genesis

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Summary: Insufficiency of surfactants is a core factor in respiratory distress syndrome, which causes apnea and neonatal death, particularly in preterm infants. Surfactant proteins are secreted by alveolar type II cells in the lung epithelium, the differentiation of which is regulated by Fgf10 elaborated by the adjacent mesenchyme. However, the molecular regulation of mesenchymal Fgf10 during lung development has not been fully understood. Here, we show that Pbx1, a homeodomain transcription factor, is required in the lung mesenchyme for the expression of Fgf10. Mouse embryos lacking Pbx1 in the lung mesenchyme show compact terminal saccules and perinatal lethality with failure of postnatal alveolar expansion. Mutant embryos had severely reduced expression of Fgf10 and surfactant genes (Spa, Spb, Spc, and Spd) that are essential for alveolar expansion for gas exchange at birth. Molecularly, Pbx1 directly binds to the Fgf10 promoter and cooperates with Meis and Hox proteins to transcriptionally activate Fgf10. Our results thus show how Pbx1 controls Fgf10 in the developing lung. genesis 52:399-407, 2014. V C 2014 Wiley Periodicals, Inc.

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Section: Introductionmentioning

confidence: 99%

Pbx1 activates Fgf10 in the mesenchyme of developing lungs

Lin

Shang

et al. 2014

Genesis

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“…Pulmonary surfactant-associated proteins (SP) include SP-A, SP-B, SP-C and SP-D which play pivotal roles in the function and metabolism of pulmonary surfactant. SP-B is one of the important SPs and can reduce or alter the surface tension via changing the surface area, which prevents the alveolar collapse (1)(2)(3)(4)(5). Respiratory distress syndrome (RDS) is a multifactorial and multi-gene disease, and a genetic factor has been found to be involved in the pathogenesis of RDS.…”

Section: Introductionmentioning

confidence: 99%

Expression of Pulmonary Surfactant-Associated Protein B in Neonatal Respiratory Distress Syndrome

Yin¹,

Wang²,

Xie³

et al. 2013

Journal of Medical Biochemistry

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SummaryBackground: The aim of this study was to investigate the role of pulmonary surfactant-associated protein B (SP-B) expression in the pathogenesis of neonatal respiratory distress syndrome (RDS) via detecting the protein and mRNA expression of SP-B. Methods: A total of 60 unrelated neonates who died of RDS were chosen as the RDS group and then subgrouped into ≤32 weeks group, 32∼37 weeks group and ≥37 weeks group (n=20). Sixty neonates who died of other diseases were enrolled as controls and subdivided into 3 matched groups based on the gestational age. Western blot assay and RT-PCR were employed. Results: In the RDS group, SP-B protein expression was reduced or deficient in 8 neonates of which 6 had no SP-B protein expression. In the control group, only 1 had reduced SP-B protein expression. The reduced or deficient SP-B protein expression in 9 neonates of both groups was noted in the ≥37 weeks group. In the RDS group, the SP-B mRNA expression was significantly lower than that in the control group. In the ≤37 weeks group, SP-B mRNA expression was comparable between the RDS group and control group. In the 32∼37 weeks group, the SP-B mRNA expression in the RDS group was significantly reduced when compared with the control group. In the ≥37 weeks group, the SP-B mRNA expression in the RDS group was dramatically lower than that in the control group. Conclusions: Alteration of SP-B expression is present at transcriptional and translational levels. Reduction of SP-B mRNA and protein expression is involved in the pathogenesis of RDS.

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“…6 Deficiency or dysregulation of SP-A is implicated in developmental pulmonary pathologies including respiratory disease syndrome of prematurity and its sequelae of chronic lung disease or bronchopulmonary dysplasia. [7][8][9] Interestingly, these molecules have been found in organs other than those in the pulmonary system. [10][11][12] SP-A has been identified in vaginal and amniotic fluid, synovial fluid, the gastrointestinal tract, and the ocular surface, 13 thus broadening the possible function of these immunity-regulating proteins.…”

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confidence: 99%