New paper out!

The cerebellum is not only responsible for motor and sensory functions; it has recently been shown that it is also involved in our social behavior. In a study published in the journal Nature Neuroscience, in which Dr Laura Cutando is the first author, researchers analyzed how it occurs in mice and found out that dopamine is the neurotransmitter in charge of this effect.


Until recently, emotions and our social behavior were thought to be regulated primarily in a set of brain areas called the limbic system through a neurotransmitter called dopamine. This region is where the processes of motivation and reward take place. However, several recent studies have suggested that the cerebellum, a region primarily involved in motor control, also plays an important role, although it was not clear how this effect occurred. In this study, in which also participated Dr Emma Puighermanal and Dr Albert Quintana, they show that, contrary to what was thought, there are type 2 dopamine receptors (also called D2) in mice cerebellum, and they are those modulating, in this area of the brain, social aspects of behavior.

Through various techniques, such as histological analysis, the study of cell RNA, or the observation of 3D imaging, researchers have observed that a group of cells in the cerebellum, called Purkinje cells, present D2 receptors. “This is a key finding, because it was thought that the existence of such receptors in this area was almost non-existent”, explains Dr Emmanuel Valjent, researcher at INSERM (France) and coordinator of the article.


To study their function, genetic editing techniques have been used to overexpress or remove D2 receptors in Purkinje cells from the cerebellum of adult mice. Then, they analyzed how these animals interact with other unknown mice. “We have demonstrated, through behavioral tests, that D2 modulates social interaction. Also, we did not notice any difference in the ability of these animals to perform motor tasks or coordinate movements, so these functions would be controlled by other receptors”, explains Dr Cutando.

This study has been carried out thanks to the multidisciplinary collaboration of INSERM (France), the University of Lausanne (Switzerland), and our research group at the INC-UAB. And it is key for understanding mental disorders in which social behavior is altered; diseases for which often it is difficult to find treatments that improve the quality of life of patients and their relatives.

Read the article (


Last Tuesday, some members of the ENACH Association visited us.

(From left to the right: ENACH Association members: Tony Moreno and Naza Martínez with their daugthers, Inés and María, together with Antonio López; and, representing INc-UAB: Dr. Jesús Giraldo (INc-UAB Director) Albert and Eli)

We are going to start a new research project, promoted by them, to identify changes in the gene expression of ENACH (NBIA in English) patients’ fibroblasts. NBIA are neurodegenerative diseases in which there is an accumulation of iron in the brain. The objective of the project, framed in the ENACH Synergy Platform, is to find therapeutical targets to develop new drugs for their treatment. Research will be developed using biological material from PKAN, PLAN and BPAN patients.

It was a pleasure to have them around and we are very glad to have the chance to contribute searching solutions for these diseases.


Our project has been selected by La Marató 2019

Our research project ‘Modulation of mitochondrial retrograde signaling as a treatment for Leigh syndrome’ has been selected by La Marató de TV3 Foundation, within its 2019 edition, dedicated to rare diseases, to receive 300,000 euros of funding.

It is a collaboration between Dr. Francesc Xavier Soriano’s lab and ours, in which we are going to study mitochondrial retrograde signaling (RTM), a mechanism neurons use to compensate for alterations produced by mitochondrial dysfunctions. Potentiating RTM through drugs as CBD is an interesting therapeutical approach for mitochondrial disease, but we still have to better understand how RTM works to find these drugs that can have the best effectiveness.

We are really glad to have this opportunity!

We try to stay safe and help as much as we can!

These are difficult days all around the world. Here in Barcelona, the number of infected people is still increasing, with no prospects for improvement in the short term. Hospitals are facing a lack of human and material resources, hence our University made a call to gather protection and detection equipment. Among other material, they collected around 90000 gloves and 4000 masks. Great job!

In the Quintana lab we are trying to help as much as we can, donating protection equipment and loaning an extraction station to Parc Taulí Hospital. This machine is an automatic processor for obtaining nucleic acids, that can be used for the diagnose of CO-VID19.  Also, some people in the lab offered their hands to help doing PCR diagnosis if the Health Department needed it.

We stay at home, but try our best to continue with the scientific activity. Today we did our first long distance lab meeting, pyjamas allowed:

We hope everything will get better soon, and all of you will be okay!

Take care <3

The VII INc Scientific Conferences

At the beginning of the month, the Institut de Neurociències celebrated its 7th Scientific Conferences, and we were -obviously- there!

The event was held in Sant Feliu de Guíxols, by the sea, in the nicest hotel, and gathered together more than 120 students and institute members.

Albert was the conference coordinator and worked very hard for everything to be perfect.

There were more than 30 talks, poster sessions and time for having fun, as well:

(Please, take your time to observe this picture of the costume contest, don’t miss any details…)

Several members of the group participated in the conferences: Elisenda gave a talk on the first day regarding new techniques we are using, Patrizia explained the research she is currently carrying out, and Emma chaired a scientific session. Also, Andrea, Laura, Pablo, Pati and Patrizia presented a poster.

On the fun side, Abel organized an amazing trivia game, on the first night, that Laura’s team won. Pablo’s team won the costume contest, dressing as Disney characters; and Albert and Eli won the partying contest, almost closing the disco.

They were great days with great people! Already looking forward next year’s edition!

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!

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.


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…

VI INc Scientific Conferences

Last 10th and 11th of October, the INc celebrated its VI Scientific Conferences, and we were obviously there. It was fantastic! More than 130 researchers and students gathered together in a beautiful hotel, in Sant Feliu de Guíxols, to present their research and foster collaborations.

PhD students of 2nd year gave scientific communications explaining their projects, and Andrea was one of them. Pablo gave a talk as well, and Irene, Álex, Fabien, Pati and Laura presented posters. It was a great opportunity for them to receive interesting comments about their work.

Dr. Carmen Sandi, president of the Federation of European Neuroscience Societies (FENS), attended the conferences and gave an amazing plenary lecture. It was a pleasure to have the chance to talk to her!

After the plenary lecture, we all played a Trivia game, and guess what team was winning! Albert, Elisenda, Irene and Álex; ‘The Tsunamis’. Then, some technological issues happened… A conspiracy?

The conferences were also a good opportunity for us to better know some companies that provide services related to our work: publishing houses, lab material suppliers, etc. We would like to thank all of them for sponsoring the conferences!

Even knowing all the stress involved in the organization, we are already looking forward to the next edition of the conferences. Hopefully, the sun will rise to the occasion next time!

(The sun was only there for this picture…)

We will receive an ERC Proof of Concept Grant!

The European Research Council (ERC) has selected us for a Proof of Concept grant to develop a new research line focused on a novel approach to figh antibiotic resistances: the ResisTEST. This project, derived from the knowledge and experience gathered with NEUROMITO, has been chosen for its innovation potential, its significant impact and its quality, and will receive an amount of 150,000€ for a 18 month period. The objective is to bring closer to the market a new tool to help face one of the most important challenges that society will have to face within the next years: the antibiotic crisis. We are very glad to have this opportunity!


Research: Altered protein modifications in mitochondrial disease

Our latest paper is out!

In collaboration with the Frizzell lab at the University of South Carolina we have published the article titled:

Succination is increased on select proteins in the brainstem of the Ndufs4 knockout mouse, a model of Leigh syndrome.” 

in the journal Molecular and Cellular Proteomics.

In this paper, we have been able to identify that the metabolic deficits mediated by mitochondrial dysfunction (we used our Leigh Syndrome mice for this study) lead to permanent modifications in discrete proteins in affected neurons.

These modifications (called succination) have been shown to impair the activity of proteins in other studies, therefore we believe these mitochondrial proteins will also lose function in our model. Interestingly, our study shows that these modifications are only observed in cells residing in areas affected by the pathology, further enhancing the idea that they may mediate the selective damage observed in mitochondrial disease.

The main proteins observed to be altered, VDAC1 and VDAC2, are key factors controlling transport of ions and molecules inside the mitochondria, so these results open a new and interesting line of research in the lab in the overarching goal of finding a cure for mito disease.




A busy couple of months!

It’s been a while since the last post, and for a good reason!

It has been a really hectic couple of months…

But first things first…

During these months the lab space has taken shape, there is still some construction going on, but the main lab space is done. You can take a look at the benches before (and after) adding all the equipment.


Importantly, our lab name plate is here! it is so cool to have it already in place, now we are even more excited to do science!

lab tag


The second remarkable event for the lab (other than the summer break) has been attending, in September, to the EMBO  workshop “Mitochondrial DNA and degeneration” held in Sitges, Barcelona.

It was a great meeting with plenty of exciting and eye-opening talks that showed us the cutting-edge science in mitochondrial research. Plus, we met many excellent mitochondrial scientists and potential collaborations!

FOTO GRUPO 1 psd (2)

Can you find Waldo (a.k.a the Quintana lab members?)

And last, but not least, we are really excited to introduce our new lab members 🙂 We welcome our first graduate students, Pati and Pablo, but this really deserves its own post!


The lab gets bigger but we all share our drive to make excellent science in mitochondrial disease research!


The Quintana lab goes across the pond…

It has been an eventful couple of months for the Quintana lab.

It all started when we received a great offer to locate the lab at the Institute of Neurosciences at the Universitat Autonoma de Barcelona, in the outskirts of Barcelona, Spain and have our research funded by one of the prestigious ERC Starting Grants (NEUROMITO: more in current projects).

ercAfter some careful thought, we have decided that this is a great opportunity for the lab to grow and to pursue our goal of finding a cure for mitochondrial disease. This funding has allowed most of the lab to move to Barcelona and will sustain and enhance our research capability in the future.

Even though it is sad to leave Seattle and to say goodbye to our friends at Seattle Children’s Research Institute and the Northwest Mitochondrial Research Guild, we believe that this will be a positive move and that undoubtedly we will continue collaborating towards our common goal. We will always be in debt for the great opportunity, training and mentoring provided by our experience and colleagues in Seattle and we will continue to grow in Barcelona.

All “travelling” members: me (Albert), Eli, Alex, Irene and Kelsey are really looking forward to set our experiments up in Barcelona and we promise new and exciting updates soon! We are sad to leave Jessica and Ben behind, but we are sure they will have a bright and promising career in the future!

So, after a few years… we have to say: farewell Seattle and Welcome Barcelona!




Our latest paper is out!

We are really excited that our latest paper, in collaboration with the Bellen lab at Baylor, has been published in the prestigious journal Cell.

Our work, titled: Glial lipid droplets and ROS induced by mitochondrial defects promote neurodegeneration, has identified a conserved mechanism that leads to neuronal death after mitochondrial defects.

In this study, the Bellen lab, using the fruit fly as a model, identified that mitochondrial mutations causing reactive oxygen species (aka oxidative stress) induced  the accumulation of lipid droplets in glia, the cells that surround and support neurons, via activation of a pathway known as JNK/SREBP. Lipid droplets are energy storage organelles, especially when neurons are faulty, but when these lipid droplets become peroxidated glia is unable to support neurons, leading to their demise.

graphical abstract final

Credit: CellPress (

Our work was key in identifying that this mechanism was present in mice, suggesting it has been evolutionary conserved, highlighting its potential importance.

Finally, we used a potent antioxidant, AD4, that crosses the blood-brain-barrier (which limits the access of many drugs to the brain), and showed that it was able to reduce and delay the onset of the disease.

We are really excited of the future therapeutic potential of this approach and we are really happy of this fruitful collaboration (pun intended!).

The article can be accessed here.