Aberrant fibroblast growth factor receptor 2 signalling in esophageal atresia with tracheoesophageal fistula

Spilde, Troy L.; Bhatia, Amina; Mehta, Shivani; Hembree, Mark; Preuett, Barry; Ostlie, Daniel J.; Prasadan, Krishna; Li, Zhixing; Snyder, Charles L.; Gittes, George K.

doi:10.1016/j.jpedsurg.2003.12.004

Cited by 8 publications

(6 citation statements)

References 19 publications

(13 reference statements)

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“…These studies have shown that the dorsal‐ventral patterning of the signaling molecules and transcription factors in the foregut prior to separation is important for the subsequent separation. Of note is that in Adriamycin (Ben Venue Laboratories, Bedford, OH, USA)‐treated rodents, EA/TEF develops with similar perturbation of signaling activities, although it remains to be investigated whether the patterning of the transcription factors has similarly been disrupted. In the future, coupling whole genome sequencing with chemical mutagenesis, e.g.…”

Section: Discussionmentioning

confidence: 99%

Genetic and cellular mechanisms of the formation of esophageal atresia and tracheoesophageal fistula

Jacobs

Que

2013

Dis Esophagus

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Foregut separation involves dynamic changes in the activities of signaling pathways and transcription factors. Recent mouse genetic studies demonstrate that some of these pathways interact with each other to form a complex network, leading to a unique dorsal-ventral patterning in the early foregut. In this review we will discuss how this unique dorsal-ventral patterning is set prior to the foregut separation and how disruption of this patterning affects the separation process. We will further discuss the roles of downstream targets of these pathways in regulating separation at cellular and molecular levels. Understanding the mechanism of normal separation process will provide us insights into the pathobiology of a relatively common birth defect Esophageal Atresia (EA) with/without Tracheo-esophageal Fistula (TEF).

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Section: Discussionmentioning

confidence: 99%

Genetic and cellular mechanisms of the formation of esophageal atresia and tracheoesophageal fistula

Jacobs

Que

2013

Dis Esophagus

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“…We have shown absence of FGF2RIIIb in fistula mesenchyme as well as its obligate ligand FGF7 from the fistula and treated lung [15]. In addition, mRNA levels of whole fistula are more closely related to the esophagus than the lung despite its respiratory origin [16]. Normal FGF expression is similar to BMP4, where it is restricted to the mesenchyme surrounding branch tips and negatively regulated between branches [10].…”

Section: Discussionmentioning

confidence: 86%

Faulty bone morphogenetic protein signaling in esophageal atresia with tracheoesophageal fistula

Crowley

Mehta

Hembree

et al. 2006

Journal of Pediatric Surgery

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“…Respiratory tissues exhibit specialised properties including branching morphogenesis [Hogan et al, 1997]. In the ARM, the fistula is not normally seen to branch in vivo, but it can be induced to branch when cultured in normal or respiratory mesen- chyme and in response to fibroblast growth factors [Crisera et al, 2000c;Spilde et al, 2004;Xiaomei et al, 2012]. The evidence to date suggests that the fistula may arise from a bi-potential origin which can be induced under certain conditions to establish a respiratory identity when exposed to the appropriate mesenchymal signals.…”

Section: Observations Of Phenotype Of the Modelmentioning

confidence: 99%

“…The fistula was positive for Fgf1 and Fgf10, but not Fgf7 mRNA. FGF receptor Fgf2rIIIb, to which Fgf7 and 10 obligately bind, was shown to be absent or downregulated in the fistula compared to respiratory structures [Crisera et al, 2000c;Spilde et al, 2003bSpilde et al, , 2004. These findings implicated FGFs as possible determinants of branching ability in the respective tissues.…”

Section: Fgf Signallingmentioning

confidence: 99%

Adriamycin-Induced Models of VACTERL Association

et al. 2012

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Animal models are of great importance for medical research. They have enabled analysis of the aetiology and pathogenesis of complex congenital malformations and have also led to major advances in the surgical and therapeutic management of these conditions. Animal models allow us to comprehend the morphological and molecular basis of disease and consequently to discover novel approaches for both surgical and medical therapy. The anthracycline antibiotic adriamycin was incidentally found to have teratogenic effects on rats, producing a range of defects remarkably similar to the VACTERL association of congenital anomalies in humans, providing a reproducible animal model of this condition. VACTERL association is a spectrum of birth defects which includes vertebral, anal, cardiovascular, tracheo-oesophageal, renal and limb anomalies. In recent years, adriamycin rodent models of VACTERL have provided valuable insights into the pathogenesis of this complex association, particularly in relation to tracheo-oesophageal malformations. The adriamycin rat model and adriamycin mouse model are now well established in the investigation of the morphology of faulty organogenesis and the regulation of gene expression in tracheo-oesophageal anomalies.

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Aberrant fibroblast growth factor receptor 2 signalling in esophageal atresia with tracheoesophageal fistula

Cited by 8 publications

References 19 publications

Genetic and cellular mechanisms of the formation of esophageal atresia and tracheoesophageal fistula

Genetic and cellular mechanisms of the formation of esophageal atresia and tracheoesophageal fistula

Faulty bone morphogenetic protein signaling in esophageal atresia with tracheoesophageal fistula

Adriamycin-Induced Models of VACTERL Association

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