PUBLICATION

TGFβ-facilitated optic fissure fusion and the role of bone morphogenetic protein antagonism

Authors
Knickmeyer, M.D., Mateo, J.L., Eckert, P., Roussa, E., Rahhal, B., Zuniga, A., Krieglstein, K., Wittbrodt, J., Heermann, S.
ID
ZDB-PUB-180330-3
Date
2018
Source
Open Biology   8(3): (Journal)
Registered Authors
Heermann, Stephan, Wittbrodt, Jochen
Keywords
BMP, ECM, TGFβ, coloboma, optic fissure fusion
MeSH Terms
  • Animals
  • Bone Morphogenetic Proteins/antagonists & inhibitors*
  • Coloboma/drug therapy
  • Coloboma/genetics*
  • Disease Models, Animal
  • Extracellular Matrix/metabolism
  • Follistatin/metabolism
  • Gene Expression Profiling/methods*
  • Intercellular Signaling Peptides and Proteins/metabolism
  • Mice
  • Signal Transduction
  • Transforming Growth Factor beta2/genetics*
  • Zebrafish/metabolism
  • Zebrafish Proteins/metabolism
PubMed
29593116 Full text @ Open Biol.
Abstract
The optic fissure is a transient gap in the developing vertebrate eye, which must be closed as development proceeds. A persisting optic fissure, coloboma, is a major cause for blindness in children. Although many genes have been linked to coloboma, the process of optic fissure fusion is still little appreciated, especially on a molecular level. We identified a coloboma in mice with a targeted inactivation of transforming growth factor β2 (TGFβ2). Notably, here the optic fissure margins must have touched, however failed to fuse. Transcriptomic analyses indicated an effect on remodelling of the extracellular matrix (ECM) as an underlying mechanism. TGFβ signalling is well known for its effect on ECM remodelling, but it is at the same time often inhibited by bone morphogenetic protein (BMP) signalling. Notably, we also identified two BMP antagonists among the downregulated genes. For further functional analyses we made use of zebrafish, in which we found TGFβ ligands expressed in the developing eye, and the ligand binding receptor in the optic fissure margins where we also found active TGFβ signalling and, notably, also gremlin 2b (grem2b) and follistatin a (fsta), homologues of the regulated BMP antagonists. We hypothesized that TGFβ is locally inducing expression of BMP antagonists within the margins to relieve the inhibition from its regulatory capacity regarding ECM remodelling. We tested our hypothesis and found that induced BMP expression is sufficient to inhibit optic fissure fusion, resulting in coloboma. Our findings can likely be applied also to other fusion processes, especially when TGFβ signalling or BMP antagonism is involved, as in fusion processes during orofacial development.
Genes / Markers
Figures
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Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping