Structure and epitope distribution of heparan sulfate is disrupted in experimental lung hypoplasia: a glycobiological epigenetic cause for malformation?
2011

Heparan Sulfate Changes in Lung Development and Hypoplasia

publication Evidence: moderate

Author Information

Author(s): Sophie M. Thompson, Marilyn G. Connell, Toin H. van Kuppevelt, Ruoyan Xu, Jeremy E. Turnbull, Paul D. Losty, David G. Fernig, Edwin C. Jesudason

Primary Institution: Institute of Integrative Biology, University of Liverpool

Hypothesis

Is the disruption of heparan sulfate structure a glycobiological epigenetic cause for lung malformation?

Conclusion

The study identifies significant molecular defects in hypoplastic lungs, suggesting that heparan sulfate may contribute to lung development issues in congenital diaphragmatic hernia.

Supporting Evidence

  • Heparan sulfate is crucial for airway morphogenesis.
  • Disruption of heparan sulfate biosynthesis leads to neonatal death in mice.
  • Congenital diaphragmatic hernia is associated with lung hypoplasia and pulmonary hypertension.
  • Specific heparan sulfate structures are recognized by growth factors like FGF2 and FGF9.
  • Abnormalities in heparan sulfate structure may disrupt lung development signaling.
  • Therapeutics targeting heparan sulfate could improve lung growth in congenital diaphragmatic hernia.

Takeaway

Heparan sulfate is important for lung growth, and when it doesn't work right, it can lead to problems like underdeveloped lungs in babies.

Methodology

The study used a nitrofen rat model to analyze heparan sulfate structure and distribution in normal and hypoplastic lungs using specific antibodies.

Limitations

The study primarily focuses on a single animal model and may not fully represent human conditions.

Digital Object Identifier (DOI)

10.1186/1471-213X-11-38

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