Pulmonary elastin expression is decreased in the nitrofen-induced rat model of congenital diaphragmatic hernia

Mychaliska, George B.; Officer, Susan; Heintz, Catherine; Starcher, Barry; Pierce, Richard A.

doi:10.1016/j.jpedsurg.2004.01.028

Cited by 9 publications

(7 citation statements)

References 32 publications

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“…However, it should be emphasized that in this model, lung hypoplasia and immaturity also occur in pups that do not develop CDH. Although less marked, the morphology of lungs of fetuses without hernia was reported to be similar to that of CDH lungs, including paucity of elastic fiber deposits in septa (21). In agreement with this observation, we found reduced FGF18 and tropoelastin expression in nitrofen-treated fetuses devoid of hernia.…”

Section: Treatments Restore Fgf18 Expression and Elastin Deposition Isupporting

confidence: 88%

“…Moreover, in the ovine model, alveolar hypoplasia occurred in the absence of reduction in bronchiolar generations, due to late creation of hernia (19), and discontinuous, uncondensed elastin aggregates have been described in alveolar septa (20). Decreased elastin expression with less elastin deposition and disorganized distribution, have also been reported in the nitrofen-model (21).…”

Section: Introductionmentioning

confidence: 53%

“…Contradictory observations have been reported in the nitrofen model with either decreased (21 and present data) or increased (39) tropoelastin transcripts. The reason for the discrepancy between studies is unclear, but may be due to methodological differences(21). Moreover, reduced levels of tropoelastin transcripts in the nitrofen model were corroborated by reduction of desmosine content (indicative of cross-linked elastin) in the lung ipsilateral to hernia(21).…”

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confidence: 99%

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Decreased Lung Fibroblast Growth Factor 18 and Elastin in Human Congenital Diaphragmatic Hernia and Animal Models

Boucherat¹,

Benachi²,

Barlier-Mur³

et al. 2007

Am J Respir Crit Care Med

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Section: Treatments Restore Fgf18 Expression and Elastin Deposition Isupporting

confidence: 88%

Section: Introductionmentioning

confidence: 53%

mentioning

confidence: 99%

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Decreased Lung Fibroblast Growth Factor 18 and Elastin in Human Congenital Diaphragmatic Hernia and Animal Models

Boucherat¹,

Benachi²,

Barlier-Mur³

et al. 2007

Am J Respir Crit Care Med

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“…The molecular mechanisms controlling alveolarization involve various growth factor signalings, transcriptions factors, and ECM components [17]. In human and experimental CDH, defective alveolarization has been linked to abnormal growth factors signalings, leading to inadequate elastin synthesis and deposition as well as vascular underdevelopment [6,18–21]. Although the enhancement of alveolar formation is one of the most striking known effects of TO, the transcriptional mechanisms by which TO stimulates alveolarization have not been completely elucidated.…”

Section: Introductionmentioning

confidence: 99%

Alveolarization Genes Modulated by Fetal Tracheal Occlusion in the Rabbit Model for Congenital Diaphragmatic Hernia: A Randomized Study

et al. 2013

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BackgroundThe mechanisms by which tracheal occlusion (TO) improves alveolarization in congenital diaphragmatic hernia (CDH) are incompletely understood. Therefore transcriptional and histological effects of TO on alveolarization were studied in the rabbit model for CDH. The question of the best normalization strategy for gene expression analysis was also addressed.MethodsFetal rabbits were randomized for CDH or sham operation on gestational day 23/31 and for TO or sham operation on day 28/31 resulting in four study groups. Untouched littermates were added. At term and before lung harvest, fetuses were subjected to mechanical ventilation or not. Quantitative real-time PCR was performed on lungs from 4–5 fetuses of each group with and without previous ventilation. Stability of ten housekeeping genes (HKGs) and optimal number of HKGs for normalization were determined, followed by assessment of HKG expression levels. Expression levels of eleven target genes were studied in ventilated lungs, including genes regulating elastogenesis, cell-environment interactions, and thinning of alveolar walls. Elastic staining, immunohistochemistry and Western blotting completed gene analysis.ResultsRegarding HKG expression, TO increased β-actin and β-subunit of ATP synthase. Mechanical ventilation increased β-actin and β2-microglobulin. Flavoprotein subunit of succinate dehydrogenase and DNA topoisomerase were the most stable HKGs. CDH lungs showed disorganized elastin deposition with lower levels for tropoelastin, fibulin-5, tenascin-C, and α6-integrin. After TO, CDH lungs displayed a normal pattern of elastin distribution with increased levels for tropoelastin, fibulin-5, tenascin-C, α6-integrin, ß1-integrin, lysyl oxidase, and drebrin. TO increased transcription and immunoreactivity of tissue inhibitor of metalloproteinase-1.ConclusionsExperimental TO might improve alveolarization through the mechanoregulation of crucial genes for late lung development. However part of the transcriptional changes involved genes that were not affected in CDH, raising the question of TO-induced disturbances of alveolar remodeling. Attention should also be paid to selection of HKGs for studies on mechanotransduction-mediated gene expressions.

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“…They account for 1% of all CDH patients and are much more frequently associated with bilateral PH and other major anomalies than unilateral CDH and with a higher mortality rate (65 vs. 35%) [1]. Pulmonary abnormalities in CDH include PH as well as vascular abnormalities (such as increased wall thickness), reduced lung compliance (due to alterations in the extracellular matrix and surfactant deficiency), leading to persistent pulmonary hypertension (PPH) [4][5][6][7].…”

Section: Discussionmentioning

confidence: 99%

Bilateral congenital diaphragmatic hernia and gastroschisis in a newborn: can low intrathoracic pressure prevent the pulmonary hypoplasia?

2007

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Congenital diaphragmatic hernia (CDH) is associated with high mortality and morbidity due to pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Bilateral CDH is extremely rare with poor prognosis. It is usually accepted that PH in CDH is due to the herniation of abdominal viscera in the thorax leading to compression of the lung and preventing the normal lung development. On the other hand, some authors suggest that the PH occurs independently from the intrathoracic pressure in foetuses with CDH because of embryologic and genetic factors. We report a case of a newborn with bilateral CDH and gastroschisis born without PH, with favourable outcome. We support the hypothesis that a low intrathoracic pressure in patients with CDH allows an improved lung development.

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Pulmonary elastin expression is decreased in the nitrofen-induced rat model of congenital diaphragmatic hernia

Cited by 9 publications

References 32 publications

Decreased Lung Fibroblast Growth Factor 18 and Elastin in Human Congenital Diaphragmatic Hernia and Animal Models

Decreased Lung Fibroblast Growth Factor 18 and Elastin in Human Congenital Diaphragmatic Hernia and Animal Models

Alveolarization Genes Modulated by Fetal Tracheal Occlusion in the Rabbit Model for Congenital Diaphragmatic Hernia: A Randomized Study

Bilateral congenital diaphragmatic hernia and gastroschisis in a newborn: can low intrathoracic pressure prevent the pulmonary hypoplasia?

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