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Cardiolipin as Key Lipid of Mitochondria in Health and Disease

October 1-2, 2017
Martina Franca, Italy

By Angela Corcelli, PhD, Professor of Physiology, Department of Basic Medical Sciences, Neurosciences and Sensory Organs, University of Bari Aldo Moro, Bari, Italy

Differently from the previous meetings, this time I shared the responsibility of the meeting organization not only with Michael Schlame (NY) and Simona Lobasso (Bari), but also with Peter Buetikofer professor in Bern. Peter is strongly interested in cardiolipin and its role in Trypanosoma brucei, the human parasite responsible for African trypanosomes, or sleeping sickness. The parasite is carried by the tsetse fly in sub-Saharan Africa.

About the location of this edition, as said before the choice of Martina Franca was made by taking in consideration the beauty of the town and of the country around and also in the light of passion of my friend Michael for the donkeys of Martina Franca, kind of mascot. I personally found the town very charming.

In this meeting, various aspects of physiopathology of different tissues and organs have been considered and examined through a kind of filter that allows to highlight the role of lipids in cell physiology.

Of course, the focus was on cardiolipin, a special phospholipid in mitochondria and not only.

The meeting in Martina Franca had the same format of the previous ones. 1-2 days of scientific sessions after the meeting of families of Barth boys carrying a modification in the TAZ gene encoding for a transacylase operating on cardiolipin.

When the protein encoded by TAZ gene is missing, alterations in the bioenergetics of the heart and muscles occur, seriously compromising the life of the boys.  The correct diagnosis of the disease requires to perform in parallel gene and lipid analyses; in the white blood cells of Barth boys typically a decrease of cardiolipin levels and an increase of monolysocardiolipin are observed.

Monolysocardiolipin is a phospholipid only rarely seen in cell membranes. An exception is the case of the outer membrane of a gram negative bacterium Acinetobacter baumannii; a poster of my group documented this occurence.

The Taz gene has been discovered in 1996 by Daniela Toniolo. The disease arising from the lack of tafazzin was first described by a pediatrician in Holland, Peter Barth in 1983. The study of the physiological processes can often take advantage of mutations or genetic defects that specifically affect or impair one step of complex molecular pathways. Genetic defects are indeed molecular tools in hands of physiologists. We have indeed the ambition to contribute to basic knowledge and at the same time to help in finding therapeutic solutions for Barth boys.

Few words about the scientific program. In the first day, the session "Cardiolipin in pathophysiological states" was chaired by Corrado Poggesi (Florence) in the morning and Lanfranco Corazzi (Perugia) in the afternoon. In the second day we had the session  "Molecular and Pharmacological Aspects" in two parts chaired by Peter Buetikofer and Michael Schlame.

The presentations illustrated studies on heart, muscles, brain and seminal fluid. Molecular models involving lipid organization in membranes and lipid-protein interactions have been considered. The molecular effects and pharmacological value of elamipretide (or SS31 from Stealth ) targeting cardiolipin were described. There is the hope that this molecule may work in ameliorating the conditions of Barth boys.

I would like to warmly thank Michael, Peter and Simona. 

I would like to thank all the invited speakers and chairpersons first of all for coming and but also for contributing to the meeting budget by funding their travel expenses.

I would also like to acknowledge the patronage of the Italian Society for Cardiovascular Research and the University of Salento

The meeting has been supported by Barth Italia, Barth Syndrome Foundation USA, Stealth (the company producing the SS31 peptide), Avanti Polar Lipids and by the University of Bari.

A special thank you to two persons in my group Patrizia Lopalco and Michele Dibattista, assistant professors of Physiology, for the time dedicated and their patience in helping me and Simona in the local organization.

Finally I like to thank Paola Cazzaniga, president of Barth Italia, for her efforts to make stronger the net and connections between the Barth italian families.

On the web page of the meeting (, there is now the link to a video as memory of the nice days in Martina Franca; the video was produced and edited by Eugenio Laddago as gift to Barth Italia.

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