Apr 4, 2020

Cell culture system to efficiently test candidate genes and molecular pathways implicated in congenital diaphragmatic hernias

BioRxiv : the Preprint Server for Biology
E. L. BogenschutzGabrielle Kardon

Abstract

The mammalian muscularized diaphragm is essential for respiration and defects in the developing diaphragm cause a common and frequently lethal birth defect, congenital diaphragmatic hernia (CDH). Human genetic studies have implicated more than 150 genes and multiple molecular pathways in CDH, but few of these have been validated because of the expense and time to generate mouse mutants. The pleuroperitoneal folds (PPFs) are transient embryonic structures in diaphragm development and a critical cellular source of CDH. We have developed a system to culture PPF fibroblasts from E12.5 mouse embryos and show that these fibroblasts, in contrast to the commonly used NIH 3T3 fibroblasts, maintain expression of key genes in normal diaphragm development. Using pharmacological and genetic manipulations that result in CDH in vivo, we also demonstrate that differences in proliferation provide a rapid means of distinguishing healthy and impaired PPF fibroblasts. Thus, the PPF fibroblast cell culture system is an efficient tool for testing the functional significance of CDH candidate genes and molecular pathways and will be an important resource for elucidating the complex etiology of CDH.

  • References
  • Citations

References

  • We're still populating references for this paper, please check back later.
  • References
  • Citations

Citations

  • This paper may not have been cited yet.

Mentioned in this Paper

Genetic Drift
Meiotic Cell Cycle
Study
Patterns
Genome
Genes
Acclimatization
Sample Fixation
House mice
Chromosomes, Human, Pair 2

Related Feeds

BioRxiv & MedRxiv Preprints

BioRxiv and MedRxiv are the preprint servers for biology and health sciences respectively, operated by Cold Spring Harbor Laboratory. Here are the latest preprint articles (which are not peer-reviewed) from BioRxiv and MedRxiv.

Related Papers

Seminars in Pediatric Surgery
Robin D Clugston, J J Greer
American Journal of Human Genetics
A M HolderD A Scott
Seminars in Pediatric Surgery
Dustin AmeisRichard Keijzer
Proceedings of the National Academy of Sciences of the United States of America
Mauro LongoniPatricia K Donahoe
© 2020 Meta ULC. All rights reserved