Nitrofen-induced diaphragmatic hernias in rats: An animal model

Kluth, Dietrich; Kangah, R.; Reich, Peter; Tenbrinck, R.; Tibboel, Dick; Lambrecht, W.

doi:10.1016/0022-3468(90)90190-k

Cited by 230 publications

(128 citation statements)

References 7 publications

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“…Extensive data from teratogenic and genetic animal models, as well as from Donnai-Barrow syndrome (MIM# 222448) patients with LRP2 mutations, confirm the important role of vitamin A in human diaphragm and lung development (Kluth et al, 1990;Kantarci et al, 2007). Case 3.3, with a minor form of spina bifida, has the first reported association of STRA6 mutations with a neural tube closure defect, which is a result of vitamin A metabolite deficiency in mouse models (Kastner et al, 1995).…”

Section: Resultsmentioning

confidence: 96%

Phenotypic spectrum ofSTRA6mutations: from Matthew-Wood syndrome to non-lethal anophthalmia

et al. 2009

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Matthew-Wood, Spear, PDAC or MCOPS9 syndrome are alternative names used to refer to combinations of microphthalmia/anophthalmia, malformative cardiac defects, pulmonary dysgenesis, and diaphragmatic hernia. Recently, mutations in STRA6, encoding a membrane receptor for vitamin A-bearing plasma retinol binding protein, have been identified in such patients. We performed STRA6 molecular analysis in three fetuses and one child diagnosed with Matthew-Wood syndrome and in three siblings where two adult living brothers are affected with combinations of clinical anophthalmia, tetralogy of Fallot, and mental retardation. Among these patients, six novel mutations were identified, bringing the current total of known STRA6 mutations to seventeen. We extensively reviewed clinical data pertaining to all twenty-one reported patients with STRA6 mutations (the seven of this report and fourteen described elsewhere) and discuss additional features that may be part of the syndrome. The clinical spectrum associated with STRA6 deficiency is even more variable than initially described.

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Section: Resultsmentioning

confidence: 96%

Phenotypic spectrum ofSTRA6mutations: from Matthew-Wood syndrome to non-lethal anophthalmia

et al. 2009

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“…Within this context, the potential beneficial effects of prenatal GC treatment on lung maturation have been studied in the nitrofen (2, 4-dichlorophenyl-p-nitrophenyl ether)-induced CDH rat model and the surgical sheep model for CDH. The nitrofen model, based on the teratogenic effects of the herbicide, lead to a condition in rodents very similar to that observed in human CDH, including the features of PH and PPH (11). A disturbance in maternal thyroid function as well as in fetal thyroid function has been suggested to be involved in the teratogenic effect of nitrofen.…”

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confidence: 93%

Spatial and Temporal Expression of Glucocorticoid, Retinoid, and Thyroid Hormone Receptors Is Not Altered in Lungs of Congenital Diaphragmatic Hernia

et al. 2006

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ABSTRACT:The degree of associated pulmonary hypoplasia and persistent pulmonary hypertension are major determination factors for survival in congenital diaphragmatic hernia (CDH) patients. Glucocorticoids, thyroid hormone, and vitamin A have been shown to be involved in human lung development. To determine their therapeutic potential in hypoplastic lungs of CDH patients, the temporal and spatial expression of glucocorticoid receptor, thyroid hormone receptors, retinoic acid receptors, and retinoid X receptors were evaluated in lungs of CDH patients, hypoplastic lungs from other causes, and normal lungs. As a series of supportive experiments, the expressions of these receptors were analyzed in lungs of nitrofen-induced CDH rats. Immunohistochemistry (human and rat) and in situ hybridization (rat) demonstrated no overt difference between CDH, hypoplastic, and control lungs, either in the localization nor the timing of the first expression of all analyzed receptors. The mRNA expression of each receptor was detected in all human CDH lungs by quantitative PCR. Our results suggest that, as far as receptors are concerned, hypoplastic lungs of fetuses and newborns with CDH are potentially as responsive to glucocorticoids, thyroid hormone, and retinoic acid as the lungs of normal children.

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“…This human lung hypoplasia accompanies abnormal expression of serum response factor (SRF) isoforms with impairment of SRF-mediated, ASM-specific gene expression (38). Nitrofen administration to pregnant rats leads to pulmonary hypoplasia in all pups and CDH in a proportion (18). ASM in this CDH model exhibits abnormal force generation and impaired relaxation near term (3).…”

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confidence: 98%

“…If AP and growth are closely related, we postulate that FGF-10 rescue of lung hypoplasia will "normalize" ASM activity. Fourth, the nitrofen model permits the first examination of links between growth and AP where lung hypoplasia has been generated in vivo (17,18).…”

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confidence: 99%

Peristalsis of airway smooth muscle is developmentally regulated and uncoupled from hypoplastic lung growth

Jesudason

Smith²,

Connell³

et al. 2006

American Journal of Physiology-Lung Cellular and Molecular Phys

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Nitrofen-induced diaphragmatic hernias in rats: An animal model

Cited by 230 publications

References 7 publications

Phenotypic spectrum ofSTRA6mutations: from Matthew-Wood syndrome to non-lethal anophthalmia

Phenotypic spectrum ofSTRA6mutations: from Matthew-Wood syndrome to non-lethal anophthalmia

Spatial and Temporal Expression of Glucocorticoid, Retinoid, and Thyroid Hormone Receptors Is Not Altered in Lungs of Congenital Diaphragmatic Hernia

Peristalsis of airway smooth muscle is developmentally regulated and uncoupled from hypoplastic lung growth

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