Defect in cells' antenna linked to deformed organs in zebrafish

March 09, 2018

A large part of the cells in the human body carry a small antenna on their surface, a so-called cilium, which receives signals from the cell's surroundings. Researchers have now discovered a protein, CEP128, at the base of the antenna, which regulates the antenna's ability to translate specific signals inside the cell.

In fact, the protein appears to be crucial to the development of vital organs in the body. In a new study published in the scientific journal Cell Reports a team of researchers from the University of Copenhagen and the University of Southern Denmark shows that if the gene coding for this protein is inactive it causes severe malformations in zebrafish.

'We knew that something in the cells' antennas regulated these cell signals. But we now know that this protein plays a main role in balancing the signal in such a way that the organs during the body's earliest stage of development develop correctly. At least in zebrafish, where inactivity of the gene leads to severe malformations of the heart, among other things', says Professor Lars Allan Larsen from the Department of Cellular and Molecular Medicine.

Human Cells Show Similar Effect

It is the so-called TGF-beta signalling which, the researchers have now learned, is regulated by the protein. This signalling controls a series of critical cellular processes in the body, both during the development of the foetus and in adult life.

The researchers hope that more knowledge of cell signalling may ultimately lead to treatments preventing cells during foetal development from behaving incorrectly, causing malformations of the heart and other vital organs. They have not come this far yet, though, and so far they are trying to learn more about the cellular mechanisms behind the signalling.

In the new study the researchers also demonstrate the effect in human cells. They have examined the significance of the protein to cell cultures and discovered that a lack of CEP128 causes poor signalling in the cilium, as in zebrafish.

Cooperation Between Several Areas of Expertise

The study is the result of a close cooperation between Professor [M1] Lars Allan Larsen from the Faculty of Health and Medical Sciences and Associate Professor Lotte B. Pedersen and Professor Søren Tvorup Christensen from the Faculty of Science at the University of Copenhagen as well as Professor Jens S. Andersen from the University of Southern Denmark.

It is well-known that defects in the cells' antennas can cause birth defects and diseases in adulthood, and new knowledge of the causes of these conditions is therefore important for the development of new forms of treatment for patients with cell antenna defects.

'This study is a good example of what we can achieve through cross-disciplinary cooperation. None of the three research groups would have had the facilities or expertise to succeed with this research alone', says Lotte Bang Pedersen and Søren Tvorup Christensen, who expect the group to make new discoveries about the function of cell antennas in a similar way in the future.
The project is funded by the Independent Research Fund Denmark, the Novo Nordisk Foundation, the Lundbeck Foundation and the UCPH Excellence Programme for Interdisciplinary Research.

The scientific article 'CEP128 Localizes to the Subdistal Appendages of the Mother Centriole and Regulates TGF-beta/BMP Signaling at the Primary Cilium' has been published in Cell Reports.

University of Copenhagen The Faculty of Health and Medical Sciences

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