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Gewählte Publikation:

SHR Neuro Krebs Kardio Lipid

Friedmacher, F; Fujiwara, N; Hofmann, AD; Takahashi, H; Gosemann, JH; Puri, P.
Evidence for decreased lipofibroblast expression in hypoplastic rat lungs with congenital diaphragmatic hernia.
Pediatr Surg Int. 2014; 30(10):1023-1029
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Autor/innen der Med Uni Graz:
Friedmacher Florian

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Plum Analytics:
Pulmonary hypoplasia (PH) is a serious condition in newborns with congenital diaphragmatic hernia (CDH). Lipid-containing interstitial fibroblasts (LIFs) play an essential role in fetal lung maturation by stimulating alveolarization and lipid homeostasis. In rodents, LIFs are first evident during the canalicular phase of lung development with a significant increase over the last 4 days of gestation. Adipocyte differentiation-related protein (ADRP), a functional lipogenic molecular marker characterizing LIFs, is highly expressed in fetal lungs during this critical time period. We hypothesized that LIF expression in hypoplastic rat lungs is decreased in the nitrofen-induced CDH model, which is accompanied by reduced alveolar ADRP expression and lipid content. On embryonic day 9.5 (E9.5), time-mated rats received either nitrofen or vehicle. Fetuses were sacrificed on selected time points E18.5 and E21.5, and dissected lungs were divided into controls and CDH-associated PH. Pulmonary gene expression levels of ADRP were determined by quantitative real-time polymerase chain reaction. ADRP immunohistochemistry and oil red O staining were used to assess pulmonary protein expression and lipid content. Immunofluorescence double staining for alpha smooth muscle actin, which is known to be absent in LIFs, and lipid droplets was performed to evaluate the pulmonary expression of this specific subset of fibroblasts. Relative mRNA expression of ADRP was significantly reduced in lungs of CDH-associated PH on E18.5 and E21.5 compared to controls. ADRP immunoreactivity and lipid staining were markedly diminished in alveolar mesenchymal cells of CDH-associated PH on E18.5 and E21.5 compared to controls. Confocal laser scanning microscopy demonstrated markedly decreased LIF expression in alveolar interstitium of CDH-associated PH on E18.5 and E21.5 compared to controls. Decreased pulmonary LIF expression during late gestation suggests impaired LIF functioning in the nitrofen-induced CDH model, which may cause disruption in fetal alveolarization and lipid homeostasis, and thus contribute to the development of PH.
Find related publications in this database (using NLM MeSH Indexing)
Animals -
Disease Models, Animal -
Female -
Fetal Development - genetics
Fibroblasts - metabolism
Gene Expression - genetics
Gene Expression Regulation, Developmental - genetics
Hernias, Diaphragmatic, Congenital - genetics
Lung - abnormalities
Lung - embryology
Lung - metabolism
Membrane Proteins - genetics
Organogenesis - genetics
Perilipin-2 -
Pregnancy -
Rats -
Rats, Sprague-Dawley -
Real-Time Polymerase Chain Reaction - methods

Find related publications in this database (Keywords)
Adipocyte differentiation-related protein
Pulmonary hypoplasia
Congenital diaphragmatic hernia
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