The inner workings of the Quintana lab

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We received a CaixaImpulse grant

We are very glad to announce that our MitoCBD project has been selected for a CaixaImpulse grant.

MitoCBD consists in investigating the use of Cannabidiol (CBD), a substance obtained from the cannabis plant, for the treatment of mitochondrial disease.

It has been proved that CBD has anti-inflammatory and neuroprotective properties, and it was also found to restore deficits and prolong life expectancy in a mitochondrial disease model.

The aim of this project is to test the effectiveness of the CBD in other preclinical models of mitochondrial disease, with the final objective of finding a treatment for these devastating rare diseases.

There has only been 21 projects selected from all around the UE, so we are veeeery happy that MitoCBD is one of them!

 

New article!

We have a new article! Elife published our study in Leigh Syndrome, the most common mitochondrial disease with affectation of the central nervous System. In this disease, there are two brain regions that are particularly compromised: the brainstem (that controls all basic functions that keep us alive) and the basal ganglia, involved in refining motor coordination. In our study, we wanted to explore the role the protein Ndufs4 has in this affectation.

Ndufs4 is a subunit of the Complex I. It is located in the mitochondrial membrane and is involved in the respiratory chain. We knew that animal models lacking this subunit in all their cells reproduce the classical signs of Leigh Syndrome, but do symptoms appear due to all cells in the body not working properly, or some specific cells are responsible for them?

To study this, we selectively inactivate the gene codifying the protein Ndufs4 in three neuronal populations we suspected could be key, and keep it working in all other cells in the body. Observing what symptoms remained we could know the role of these neurons in the alterations these patients suffer.

What we found was that the inactivation of this subunit in a certain kind of excitatory neurons (the fancy-scientist name is vlgut2-expression glutamatergic neurons) caused brainstem inflammation, motor and respiratory deficits, and early death; and that its inactivation in inhibitory (GABAergic neurons) led to basal ganglia inflammation, severe refractory epileptic seizures and premature death.

These results are very important to contribute understand the underlying cellular mechanisms of Leigh Syndrome, as we identified which specific neurons are behind the brain alterations. Therefore, now we have new knowledge to try to identify which cellular aspects are failing in those cells so we can start to envisage different methods to correct them.

Congrats to all the team and collaborators!

Celebrating the 4th of July

Albert, Eli, Álex and Irene are a bit nostalgic of their lives in USA, and they celebrate every North-American festivity they can.

This time, we met at Andrea’s house in el Penedès (thank you again for offering the house) to honour the USA independence on the 4th of July.

Cold beers in the swimming pool, to fight the heat, and all this tasty food waiting to be cooked in the barbecue.

The burgers needed to be prepared. We mixed the meat while Albert and Eli were singing the US National Anthem. We also had frankfurts and pulled pork, which was extremely delicious.


After the meal, we made up a game with water balloons in the swimming-pool, and we played Dixit while Fabien was putting great music. Abel won not because he was the best but because we used his weird point-counting system.

It was a lovely day! It is great to be part of the Quintanalab 🙂

MitoTreat won the 9th edition of the Generació d’Idees Program!

Generació d’Idees is a program organized by Parc de Recerca UAB, to promote entrepreneurship among researchers. Communications, mental health, environment… They focus on a different topic in each edition, and this time, it was the turn of biotechnology.

During 17 sessions, researchers were given the tools to develop innovative solutions in the biotechnology field. They had modelling classes, mentoring sessions, etc., and then, after all this time, they presented six different projects to a board of experts. The most innovative one was selected… and guess which one was it! Yessss: MitoTreat!

MitoTreat was developed by Emma, Elisenda and Albert, and consists in using Cannabidiol, a substance found in cannabis, to treat mitochondrial disease. Cannabidiol has anti-inflammatory, anti-oxidant, analgesic, neuroprotective and anti-convulsive proprieties.

In animal models of mitochondrial diseases it showed excellent results, and now they want to start the preclinical trial in humans.

The project obtained a 1500 euro prize and 6 months of incubation at Eureka building, at Parc de Recerca, to improve the transference of the drug to the market.

Congrats!

Quintanalab-Calçotada 2019

The Quintanalab-Calçotada 2019 took place on a super sunny April day in El Priorat de Banyeres, a tiny village near Vilafranca. Thanks, Andrea, for offering the house!

It is already a tradition that the whole group gathers together one day to eat calçots with salsa romesco and have fun.

(Please, note Albert’s Salt Bae position…)

Àlex was the calçots chef. A fire was made, and he cooked the calçots with patience and expertise. He did an excellent job as they were very delicious!

After the heavy lunch, to digest, we played dodgeball. Ask Albert who won. Spoiler: not his team.

Then we drunk mojitos and talked and drunk more mojitos… It was a very nice day! Can’t wait for the Quintanalab-Calçotada 2020!

Swiss Light-sheet Microscopy Workshop

Last 24th and 25th of April, Andrea, Pablo, Elisenda and Albert went to Zurich to attend the Swiss Light-sheet Microscopy Workshop.

 

 

It was a two days meeting in which researchers from all over the world gathered together to share the observational techniques they are using. Users and developers discussed newest advances in the field and exchanged ideas.

Elisenda was telling me about these 3D glasses that transported you inside a gigantic transparent brain in which you could observe a specific neuron from all possible angles. Exciting!  Pablo is there in the picture:

 

 

They were very impressed by everything they saw, and learned a lot! Albert said it was a very productive conference and can’t wait to have the chance to try some of these techniques in the lab. He also told me they were happy they had the chance to have a typical Swiss dinner: fondue, raclette and rösti. Fabien might not agree about this being a typical Swiss dinner and not a French one…

Our first preprint!

We published our first preprint last week. For those who aren’t familiarized, a preprint is a study to be published in a Journal, that has not been reviewed yet. The aim is to make the results available as soon as possible so Science can advance faster. We are very excited about it, and very happy because it has been featured by the preprint platform Biorxiv for reaching the top 5% altmetric score within their first days after releasing it; and it has also been selected as one of the ERC Research results of the week! So cool!

But what is it about?

As you may know, dysfunctions of the mitochondrial energy-generating machinery cause a series of usually fatal diseases collectively known as mitochondrial disease. In this disease, high energy-requiring organs, like the brain, are especially affected, showing many severe symptoms such as motor alterations, respiratory deficits or epilepsy that, in many cases, can be fatal. Hence, our group is driven to better understand these pathologies, with the overarching goal of finding effective therapies to treat them.

This time, we focused on Leigh Syndrome, the most common presentation of mitochondrial disease with a predominant affectation of the central nervous system, particularly in two brain areas, the brainstem (that controls all basic functions that keep us alive) and the basal ganglia, involved in refining motor coordination. We knew that one protein in the mitochondria is critical in this disease: the Complex I subunit NDUFS4, because animal models lacking this subunit in all their cells reproduce the classical signs of Leigh Syndrome described before. However, there were some pressing questions remaining: do symptoms appear due to all cells in the body not working properly, or some specific cells are responsible for them?

To study this, we selectively inactivate NDUFS4 in three neuronal populations we suspected could be key, and keep NDUFS4 working in all other cells in the body. Observing what symptoms remained we could know the role of these neurons in the brain alterations these patients suffer.

What we found was that the inactivation of this subunit in a certain kind of excitatory neurons (the fancy-scientist name is vlgut2-expression glutamatergic neurons) caused brainstem inflammation, motor and respiratory deficits, and early death; and that its inactivation in inhibitory (GABAergic neurons) led to basal ganglia inflammation, severe refractory epileptic seizures and premature death.

Bottom line, these results are very important to contribute understand the underlying cellular mechanisms of mitochondrial disease in general, and Leigh Syndrome in particular, as we have identified which specific neurons are behind the brain lesions observed in this pathology and their specific contribution to the symptoms. Therefore, now we have new knowledge to try to identify which cellular aspects are failing in those cells so we can start to envisage different methods to correct them.