rain
Dissecting neuronal susceptibility to mitochondrial disease

Quintana Lab

WELCOME

Research

Saving neurons from mitochondrial disease one ATP at a time

The Quintana lab investigates the molecular mechanisms defining why some neuronal populations are particularly affected by mitochondrial disease, with the overarching goal of identifying novel targets that lead to improved treatments for mitochondrial disease patients.

Mitochondria are the powerhouses of the cell. Mutations that render mitochondria unable to generate energy cause a group of rare and usually fatal pathologies collectively known as mitochondrial disease. It has been estimated that 1 in 5000 children in the US will develop a mitochondrial disease. Currently, there is no cure for mitochondrial disease and the treatments available are mostly ineffective. Energy-demanding cells such as neurons are especially sensitive to mitochondrial disease, and they account for most of the clinical signs and symptoms observed in humans, such as hypotonia, ataxia, seizures and early death. However, even if every single cell in the body carries the mutation, only specific brain areas seem to be affected by the deficiency. The Quintana lab current research focuses on identifying the neuronal populations susceptible to mitochondrial disease and which mechanisms are making these neurons die. This knowledge is essential to understand and fight these incurable diseases. The Quintana lab uses a wide array of approaches, combining molecular biology, stereotaxic surgery, mouse genetics and behavior, biochemistry, histology, optogenetics and in vivo electrophysiology to reveal novel pathways and mechanisms in neuronal function and pathology and open new and unexplored lines of research and therapeutic targets to treat mitochondrial disease encephalopathy.

 

“EVERY SINGLE CELL RELIES ON MITOCHONDRIA TO SURVIVE.

1 IN 5000 CHILDREN ARE AFFECTED BY MITOCHONDRIAL DISEASE”

Current Projects

Recent Publications

Sharing our findings to advance knowledge

 

Complete Publication List (PubMed)

Public Engagement

Giving back to the community and spreading awareness of mitochondrial disease

Finding a cure for mitochondrial disease requires a constant dialogue and synergies between basic researchers, clinicians and the community. Raising public awareness and promoting education for mitochondrial disease is vital to advance in our knowledge on the pathogenesis and treatment of mitochondrial disease.

The determined efforts of countless individuals, associations and foundations,  have greatly, and positively, impacted mitochondrial research in many levels ranging from providing the perfect environment for mitochondrial researchers to thrive to promoting changes in governmental funding agencies. Among these organizations, we would like to acknowledge the invaluable contributions of the United Mitochondrial Disease Foundation (UMDF) and the North-West Mitochondrial Research Guild in promoting mitochondrial disease research and education at a national/worldwide or local level, respectively, in the USA and AEPMI and the Fundación Mencía, in Spain.

 

UMDF NW MITO GUILD AEPMI FUNDACIÓN MENCÍA

 

The Quintana lab is committed to help increase public engagement and awareness of mitochondrial disease by ensuring the spread of our research by talking to the press and providing short summaries of key research papers and other developments in our blog and gallery. Also, by participating in public seminars and by interacting within local, national and international associations in meetings and community-oriented events.

 

“RAISING PUBLIC AWARENESS,

PROMOTING RESEARCH

 CREATING SYNERGIES.

THAT IS THE PATH TO FIND THE CURE FOR MITOCHONDRIAL DISEASE”

On the media

Members

Giving our 150% to find a cure to mitochondrial disease

Albert Quintana, PhD

Albert Quintana, PhD

Principal Investigator

Ramón y Cajal Investigator
Institut de Neurociències
Dept. Biologia Cel·lular, Fisiologia i Immunologia
Universitat Autònoma de Barcelona
CV
ResearchGate

albert.quintana@uab.cat

Elisenda Sanz, PhD

Elisenda Sanz, PhD

Senior Postdoctoral Researcher

Institut de Neurociències
Dept. Biologia Cel·lular, Fisiologia i Immunologia
Universitat Autònoma de Barcelona
CV

elisenda.sanz@uab.cat

Irene Bolea, PhD

Irene Bolea, PhD

Juan de la Cierva Postdoctoral Fellow

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

irene.bolea@uab.cat

Alex Gella, PhD

Alex Gella, PhD

Marie Curie COFUND fellow

Institut de Neurociències
Universitat Autònoma de Barcelona
CV ResearchGate

alex.gella@uab.cat

Patrizia Bianchi, PhD

Patrizia Bianchi, PhD

Postdoctoral researcher

Institut de Neurociències
Universitat Autònoma de Barcelona

patrizia.bianchi@uab.cat

Fabien Menardy, PhD

Fabien Menardy, PhD

Postdoctoral researcher

Institut de Neurociències
Universitat Autònoma de Barcelona

fabien.menardy@uab.cat

Emma Puighermanal, PhD

Emma Puighermanal, PhD

Post-doctoral researcher

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

emma.puighermanal@uab.cat

Patricia Prada

Patricia Prada

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona

patricia.prada@uab.cat

Pablo Machuca

Pablo Machuca

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

pablo.machuca@uab.cat

Andrea Urpí

Andrea Urpí

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

andrea.urpi@uab.cat

Laura Sánchez

Laura Sánchez

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona

laura.sanchez.benito@uab.cat

Isabella Appiah

Isabella Appiah

Lab technician

Institut de Neurociències
Universitat Autònoma de Barcelona

isabella.appiah@uab.cat

Adán Domínguez

Adán Domínguez

Undergraduate student

Institut de Neurociències
Universitat Autònoma de Barcelona

adan.dominguez@e-campus.uab.cat

Roser Bastida

Roser Bastida

Communications technician

Institut de Neurociències
Universitat Autònoma de Barcelona

roser.bastida@uab.cat

Albert Quintana, PhD

Albert Quintana, PhD

Principal Investigator

Ramón y Cajal Investigator
Institut de Neurociències
Dept. Biologia Cel·lular, Fisiologia i Immunologia
Universitat Autònoma de Barcelona
CV
ResearchGate

albert.quintana@uab.cat

Elisenda Sanz, PhD

Elisenda Sanz, PhD

Senior Postdoctoral Researcher

Institut de Neurociències
Dept. Biologia Cel·lular, Fisiologia i Immunologia
Universitat Autònoma de Barcelona
CV

elisenda.sanz@uab.cat

Irene Bolea, PhD

Irene Bolea, PhD

Juan de la Cierva Postdoctoral Fellow

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

irene.bolea@uab.cat

Alex Gella, PhD

Alex Gella, PhD

Marie Curie COFUND fellow

Institut de Neurociències
Universitat Autònoma de Barcelona
CV ResearchGate

alex.gella@uab.cat

Patrizia Bianchi, PhD

Patrizia Bianchi, PhD

Postdoctoral researcher

Institut de Neurociències
Universitat Autònoma de Barcelona

patrizia.bianchi@uab.cat

Fabien Menardy, PhD

Fabien Menardy, PhD

Postdoctoral researcher

Institut de Neurociències
Universitat Autònoma de Barcelona

fabien.menardy@uab.cat

Emma Puighermanal, PhD

Emma Puighermanal, PhD

Post-doctoral researcher

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

emma.puighermanal@uab.cat

Patricia Prada

Patricia Prada

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona

patricia.prada@uab.cat

Pablo Machuca

Pablo Machuca

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

pablo.machuca@uab.cat

Andrea Urpí

Andrea Urpí

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona
CV

andrea.urpi@uab.cat

Laura Sánchez

Laura Sánchez

Graduate Student

Institut de Neurociències
Universitat Autònoma de Barcelona

laura.sanchez.benito@uab.cat

Isabella Appiah

Isabella Appiah

Lab technician

Institut de Neurociències
Universitat Autònoma de Barcelona

isabella.appiah@uab.cat

Adán Domínguez

Adán Domínguez

Undergraduate student

Institut de Neurociències
Universitat Autònoma de Barcelona

adan.dominguez@e-campus.uab.cat

Roser Bastida

Roser Bastida

Communications technician

Institut de Neurociències
Universitat Autònoma de Barcelona

roser.bastida@uab.cat

ALONE WE CAN DO SO LITTLE,

TOGETHER WE CAN DO SO MUCH

Helen Keller

Alumni

Once Quintana lab, always Quintana lab

Abel Eraso

Abel Eraso

Post-doc researcher

Institut de Neurociències
Universitat Autònoma de Barcelona

abel.eraso@uab.cat

Kelsey Montgomery

Kelsey Montgomery

Research Technician

Alumni 2014-2015
Seattle Children’s Research Institute

Alumni 2016-2018
Universitat Autònoma de Barcelona

Teresa Ximelis

Teresa Ximelis

Master Student

Alumni 2016-2017
Universitat Autònoma de Barcelona

Jessica Hui

Jessica Hui

Undergraduate Student

Alumni 2013-2015
Seattle Children’s Research Institute

Benjamin Bauer

Benjamin Bauer

Undergraduate Student

Alumni 2014
Seattle Children’s Research Institute

Abel Eraso

Abel Eraso

Post-doc researcher

Institut de Neurociències
Universitat Autònoma de Barcelona

abel.eraso@uab.cat

Kelsey Montgomery

Kelsey Montgomery

Research Technician

Alumni 2014-2015
Seattle Children’s Research Institute

Alumni 2016-2018
Universitat Autònoma de Barcelona

Teresa Ximelis

Teresa Ximelis

Master Student

Alumni 2016-2017
Universitat Autònoma de Barcelona

Jessica Hui

Jessica Hui

Undergraduate Student

Alumni 2013-2015
Seattle Children’s Research Institute

Benjamin Bauer

Benjamin Bauer

Undergraduate Student

Alumni 2014
Seattle Children’s Research Institute

LAB OPENINGS

There are no current openings in the lab at this moment.

 

Gallery

A taste of all our activities and events is just one click away

Social

Thanksgiving 2019

Costumes night at INc Scientific Conferences 2019

Celebrating the 4th of July (2019)

Celebrating the 4th of July (2019)

Calçotada 2019

Thanksgiving 2018

When we won the HallowINc contest!

 

Outreach activities at INc

Elisenda explaining the synapse at Olivereta school

 

Fabien talking about what he does in the lab at the Open House Day 2019

 

Albert probably making jokes at the Open House Day 2019

 

Álex at the Open House Day 2018

 

Andrea and Patrizia talking about Mitochondrial disease at La Fira de la Recerca en Directe

 

Andrea and Patrizia at La Fira de la Recerca en Directe

 

Laura at the Open House Day 2019

 

Neurons

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A Golgi-Cox staining of mouse cerebellum… 100-year old technique providing amazing labeling of brain cells

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A closeup of a cerebellar Purkinje neuron in an Ndufs4KO mice

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Microglial (green) and astroglial (red) reactivity observed in the vestibular and cerebellar lesions of an Ndufs4KO mouse

Triple GFAP IBA1

Microglial (green) and astroglial and radial glial (red) activation in the cerebellum of an Ndufs4KO mouse.

Activated (green) and phagocytic (red) microglial cells surround a lesion in the vestibular nucleus of an Ndufs4KO mice.

Activated (green) and phagocytic (red) microglial cells surround a lesion in the vestibular nucleus of an Ndufs4KO mice.

 

Mitochondria

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Red fluorescent mitochondria waiting to be purified. Cell nuclei in blue.

Green fluorescent mitochondria in a HEK cell

Green fluorescent mitochondria in a HEK cell

23_ol_cropped

A close-up of red fluorescent mitochondria in a HEK cell

Outreach activities in Seattle: Mito Science Night

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IMG_0503

Eli demonstrating how to fluorescently label mitochondria

IMG_0507

Nicole explaining green fluorescent worms

photo 1

Bernhard demonstrating how the Seahorse analyzes mitochondrial respiration

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A fuzzy stormtrooper mouse is discovering optogenetics

IMG_0496

Jill and Ed are fully gowned for surgery!

IMG_0494

We are ready! 🙂

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Christian is ready to show how an electrophysiological trace looks!