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Childhood ILD
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
There is a spectrum of disorders comprising acinar dysplasia and alveolar-capillary dysplasia. Typically these present in a term baby who requires positive pressure ventilation soon after birth and progresses rapidly despite every attempted treatment into terminal respiratory failure. The histological pattern is of disrupted alveolar and capillary growth, with failure of normal apposition of capillaries and airspaces. Mutations in FOXF1 and STRA6 have been found in some patients (19,20). ‘Alveolar-capillary dysplasia with misalignment of pulmonary veins’ is in fact a misnomer; the ‘misaligned’ pulmonary veins have been shown by elegant morphometric studies to be dilated bronchial veins, with the anastomotic communications with the pulmonary venous system and distal pulmonary veins absent or obliterated (21).
Biliary atresia
Published in Prem Puri, Newborn Surgery, 2017
Furthermore, at this stage, the only biliary structure is the extrahepatic bile and emerging gallbladder. This appears at about day 20 as an outpouching from the distal foregut and develops into a funnel-shaped structure with a lumen and a gallbladder evident by day 45. It is lined by cholangiocytes derived from foregut endoderm, expressing transcription factors common to the pancreas and duodenum (e.g., PDX-1, PROX-1, HNF-6). The molecular mechanisms regulating this phase of biliary development are not well described in humans, but mice deficient in Pdx-119 or Hes1 (a Notch-dependent transcription factor), Hnf-6, Hnf-1β, or Foxf1 (a transcription factor target for Sonic Hedgehog signaling) can show altered development of the gallbladder with a normal common bile duct (CBD).14
Integrated Analysis of lncRNAs and mRNAs Identifies a Potential Driver lncRNA FENDRR in Lung Cancer in Xuanwei, China
Published in Nutrition and Cancer, 2021
Yong Duan, Wen-Xing Li, Yan Wang, Ying Zhao, Jie Shen, Cheng-Jun Deng, Qing Li, Ran Chen, Xiao Liu, Yan-Liang Zhang
We used the above consistently deregulated mRNAs and lncRNAs to construct the co-expression network (Figure 3). The five down-regulated lncRNAs were positively correlated with one or more mRNAs (except SIX1). In the network, lncRNA ENSG00000257424 interacted with 12 mRNAs, lncRNA FENDRR interacted with 11 mRNAs, KLF4, LOC101928370, and CLEC3B interacted with all five lncRNAs. Among these genes, KLF4 encodes a protein that belongs to the Kruppel family of transcription factors which play important roles in cell cycle and DNA replication. SDPR encodes a calcium-independent phospholipid-binding protein that is a substrate for protein kinase C (PKC) phosphorylation. FOXF1 belongs to the forkhead family of transcription factors and play a role in DNA binding and transcription.
Chronic interstitial lung diseases in children: diagnosis approaches
Published in Expert Review of Respiratory Medicine, 2018
Nadia Nathan, Laura Berdah, Keren Borensztajn, Annick Clement
Diffuse developmental disorders are severe and often lethal conditions [61]. Acinar dysplasia and congenital alveolar dysplasia are characterized by an arrested development at the pseudoglandular or canalicular/saccular stages, respectively. Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) associates an aberrant parenchymal development of the lung with thickened interstitium, a poor capillary bed, and in most cases, the presence of pulmonary veins in the bronchovascular axis instead of at the periphery of the lobule. The newborns present with refractory hypoxemia and severe pulmonary hypertension. The diagnosis is usually made on autopsies. Recently, mutations in TBX4 and FGFR2 have been associated with syndromic acinar dysplasia [62,63]. The majority of the described cases of ACD/MPV have been found to be related to FOXF1 mutations or to mutations in the FOXF1 enhancer. Extra-pulmonary disorders are documented in more than half of the cases: congenital heart diseases, digestive malformations or malrotations, and genitourinary malformations [64–66].
FOXF1 ameliorates angiotensin II-induced cardiac fibrosis in cardiac fibroblasts through inhibiting the TGF-β1/Smad3 signaling pathway
Published in Journal of Receptors and Signal Transduction, 2020
Forkhead Box F1 (FOXF1), a member of the FOX family, has been found to be involved in the regulation of fibrogenesis and expressed in multiple mesenchyme-derived cell types, including normal hepatic stellate cells, peribronchial smooth muscle cells, lung microvascular endothelial cells, and fibroblasts [6]. In the advanced disease state of hepatocellular carcinoma, which is associated with significant fibrotic depositions, FOXF1 expression has been shown to be significantly decreased [7]. FOXF1 inhibits pulmonary fibrosis by regulating a switch from N-cadherin to Cadherin-11 in lung myofibroblasts. However, the role of FOXF1 in cardiac fibrosis remains unknown. In this study, we used Ang II to induce an in vitro model of cardiac fibrosis in CFs.