Rosa Barrio
rbarrio
Rosa Barrio
GROUP LEADER
Ubiquitin-likes And Development Lab
Address: Bizkaia Science and Technology Park, building 801A, Derio (Bizkaia)

Rosa Barrio obtained her BSc in Biology from the Autonomous University of Madrid (UAM) and her PhD in Sciences from the same University.

Dr. Barrio joined the laboratory of Prof. FC Kafatos at Harvard University (Cambridge, USA), IMBB (Greece), and EMBL (Germany) to study SALL transcription factors, which mutations contribute to hereditary human diseases such as Townes-Brocks Syndrome (TBS) and Okihiro Syndrome (OS). In 2001, Dr. Barrio moved to the laboratory of Dr. JF de Celis and Prof. García-Bellido at CBMSO (Madrid, Spain). Her focus was on understanding how SALL factors contribute to development. In 2003, she was awarded a prestigious Ramón y Cajal contract.

In 2004, Dr. Barrio joined CIC bioGUNE as a Group Leader, leading the Ubiquitin-likes And Development Lab. Her laboratory is focused on understanding protein homeostasis regulation, especially focusing on the Ubiquitin-like (UbL) family. The group develops technologies like BioUbL, SUMO-ID, and BioE3 to identify UbL modified proteins, their interactors, and E3 ligase targets. The interests of the laboratory include the study of how disruptions in protein homeostasis contribute to rare diseases like TBS and other.

Awards & recognitions
"Incentivación de la Incorporación e Intensificación de la Actividad Investigadora (Programa I3)" awarded by the Spanish Ministry of Science and Innovation (2007). "Ramón y Cajal Program" contract awarded by the Spanish Ministry of Education and Science (2003). Contract awarded by the Spanish Ministry of Science and Technology for "Reincorporación de Doctores y Tecnólogos” (2001). Postdoctoral Fellowship granted by the Autonomous Community of Madrid (2001). Short-term EMBO Fellowship (2000). Postdoctoral Fellowship awarded by the Spanish Ministry of Education and Science (1996). Postdoctoral Fellowship granted by the Marie Curie "Human Capital and Mobility Program" (1993). Fellowship from the Rich Foundation (1993). PhD Fellowship sponsored by the Basque Government (1988).

Latest Publications

2023

2022

2021

2020

Post-translational modifications (PTMs) by members of the ubiquitin-like family (UbL) are essential for cell survival and for the proper function of organisms. Cellular signaling drives conserved enzymatic pathways that add UbL modifiers to target proteins determining their fate, including changes in protein-protein interactions, localization, or stability and degradation. UbL modifications are important for proper development and maintaining homeostasis in adults. UbL-based dysfunction is implicated in many illnesses (cancer, diabetes, neurodegeneration), including rare diseases.

A low prevalence or rare disease is defined as one with a prevalence of less than 1 case per 2000 inhabitants. It is estimated that there are between 5,000 and 7,000 different rare diseases, some of which affect a small number of people around the world. However, considering the various pathologies, it is calculated that between 6 and 8% of the world population would be afflicted by any of these diseases. Therefore, an estimated 30 million Europeans suffer from various rare diseases. We are interested in the impact of protein homeostasis deregulation in rare diseases; one example is Townes-Brocks Syndrome (TBS), affecting digit formation, kidney function and hearing. We explore the role of UbL-based regulation in TBS and other low prevalence diseases.

Research line 1: Regulation of post-translational modifications by members of the ubiquitin-like family
Despite their relevance in physiology and diseases, studying UbL modifications is particularly challenging because they are normally transient and occur in a small percentage of a given protein pool. To overcome these difficulties, we develop specific strategies, including BioUbL (Sci Reports 2017), to capture and identify UbL-modified proteins using in vivo biotinylation; UbL-ID (Nat Comm 2021), to identify interactors of a given protein of interest when modified by UbLs; or BioE3 (Nat Com 2023), to identify specific targets of E3 ligases. E3 ligases give specificity to the UbL system as they recognize specific targets. There are more than 600 E3 ligases and in most of the cases, targets are largely unknown. E3s are particularly relevant as they are key players in the emerging strategies of targeted protein degradation (TPD), therefore knowing endogenous targets can inform on specificity and off-targets of designed TPD drugs.

Research line 2: Proteostasis regulation in rare diseases
We are interested in understanding the mechanisms underlying TBS and other diseases related to the UbL family (e.g. Angelman Syndrome, Opitz GBBB Syndrome). Individuals with TBS, caused by mutations in the transcription factor SALL1, show anomalies in cilia formation and function due to the interaction of mutant SALL1 proteins with centrosomal/ciliary factors (Am J Human Genet 2018; eLife 2020). We investigate the relationship of mutations with the protein homeostasis regulation of LUZP1, an interactor of mutated SALL1. We also study the relationship of other rare diseases with components of the UbL family, such as E3 ligases.

Collaborations
Arkaitz Carracedo, Juen Ereño, JuanMa Falcón and Malu Martínez-Chantar (CIC bioGUNE, Spain), Ugo Mayor and Josean Rodríaguez (UPV/EHU, Spain), Orhi Barroso Gomila (Biobizkaia, Spain), Carmen Rivas (CIMUS, Santiago de Compostela, Spain), Carles Galdeano and Jordi Jiménez (University of Barcelona, Spain), Jordi Torres (University of Lleida, Spain), Manuel S Rodríguez (CNRS, France), Alfred Vertegaal (LUMC, Netherlands), Simona Polo (IFOM, Italy), Germana Meroni (University of Trieste, Italy), Tim Clausen (IMP, Austria), Nicolas Bidère (Inserm, France), Mikko Taipale (University of Toronto, Canada).

Links
Webpage: https://www.rosabarriolab.es/

Latest Publications

SUMOylation controls Hu antigen R posttranscriptional activity in liver cancer.

Lachiondo-Ortega, Sofia; Rejano-Gordillo, Claudia M; Simon, Jorge; Lopitz-Otsoa, Fernando; C Delgado, Teresa; Mazan-Mamczarz, Krystyna; Goikoetxea-Usandizaga, Naroa; Zapata-Pavas, L Estefania; ...

Cell reports

2024-03-26

SUMOylation modulates eIF5A activities in both yeast and pancreatic ductal adenocarcinoma cells

Seoane, R; Lama-Diaz, T; Romero, AM; El Motiam, A; Martinez-Ferriz, A; Vidal, S; Bouzaher, YH; Blanquer, M; Tolosa, RM; Mewa, JC; Rodriguez, MS; Garcia-Sastre, A; Xirodimas, D; Sutherland, JD; ...

CELLULAR & MOLECULAR BIOLOGY LETTERS

2024-01-16

Biotin-based strategies to explore the world of ubiquitin and ubiquitin-like modifiers

Merino-Cacho, L; Barroso-Gomila, O; Hernandez-Sánchez, S; Ramirez, J; Mayor, U; Sutherland, JD; Barrio, R;

CHEMBIOCHEM

2024-01-05

BioE3 identifies specific substrates of ubiquitin E3 ligases

Barroso-Gomila, O; Merino-Cacho, L; Muratore, V; Perez, C; Taibi, V; Maspero, E; Azkargorta, M; Iloro, I; Trulsson, F; Vertegaal, ACO; Mayor, U; Elortza, F; Polo, S; Barrio, R; Sutherland, JD; ...

NATURE COMMUNICATIONS

2023-11-23

Structural insights into Siglec-15 reveal glycosylation dependency for its interaction with T cells through integrin CD11b

Lenza, MP; Egia-Mendikute, L; Antoñana-Vildosola, A; Soares, CO; Coelho, H; Corzana, F; Bosch, A; Manisha, P; Quintana, JI; Oyenarte, I; Unione, L; Moure, MJ; Azkargorta, M; Atxabal, U; Sobczak, ...

NATURE COMMUNICATIONS

2023-06-13

Studying the ubiquitin code through biotin-based labelling methods

Barroso-Gomila, O; Muratore, V; Merino-Cacho, L; Rodriguez, JA; Barrio, R; Sutherland, JD;

SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

2022-11-16

In memoriam: Huib Ovaa, the brain behind the formula (18/12/1973-19/05/2020)

Barrio, R; Rodriguez, MS;

SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

2022-11-16

U1A is a positive regulator of the expression of heterologous and cellular genes involved in cell proliferation and migration

Rovira, E; Moreno, B; Razquin, N; Hjerpe, R; Gonzalez-Lopez, M; Barrio, R; de los Mozos, IR; Ule, J; Pastor, F; Blazquez, L; Fortes, P;

MOLECULAR THERAPY-NUCLEIC ACIDS

2022-05-25

Editorial: 17th Spanish Society for Developmental Biology Meeting: New Trends in Developmental Biology

Araujo, SJ; Barrio, R;

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY

2022-04-20

Origin and Development of the Adipose Tissue, a Key Organ in Physiology and Disease

Parra-Peralbo, E; Talamillo, A; Barrio, R;

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY

2021-12-21

A Genotyped Case of Townes-Brocks Syndrome with Absent Pulmonary Valve Syndrome from Turkey

Ilhan, O; Gumus, E; Hakan, N; Istar, H; Harmandar, B; Olgun, H; Karakus, SC; Cullu, N; Kohlhase, J; Sutherland, JD; Barrio, R;

JOURNAL OF PEDIATRIC GENETICS

2021-12-10

Identification of proximal SUMO-dependent interactors using SUMO-ID

Barroso-Gomila, O; Trulsson, F; Muratore, V; Canosa, I; Merino-Cacho, L; Cortazar, AR; Pérez, C; Azkargorta, M; Iloro, I; Carracedo, A; Aransay, AM; Elortza, F; Mayor, U; Vertegaal, ACO; Barrio, ...

NATURE COMMUNICATIONS

2021-11-18

NEDDYLATION INHIBITION REDUCES LIVER STEATOSIS IN MAFLD MICE MODELS BY PROMOTING HEPATIC FATTY ACID OXIDATION VIA DEPTOR-mTOR AXIS

Serrano-Maciá, M; Espinosa, JS; González-Rellan, MJ; Azkargorta, M; Goikoetxea-Usandizaga, N; Lopitz-Otsoa, F; De Urturi, DS; Agudo, RR; Lachiondo-Ortega, S; Mercado-Gómez, M; Bizkarguenaga, ...

HEPATOLOGY

2021-10-01

SALL1 Modulates CBX4 Stability, Nuclear Bodies, and Regulation of Target Genes

Giordano, I; Pirone, L; Muratore, V; Landaluze, E; Pérez, C; Lang, V; Garde-Lapido, E; Gonzalez-Lopez, M; Barroso-Gomila, O; Vertegaal, ACO; Aransay, AM; Rodriguez, JA; Rodriguez, MS; Sutherland, ...

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY

2021-09-21

Neddylation inhibition ameliorates steatosis in NAFLD by boosting hepatic fatty acid oxidation via the DEPTOR-mTOR axis

Serrano-Maciá, M; Simón, J; González-Rellan, MJ; Azkargorta, M; Goikoetxea-Usandizaga, N; Lopitz-Otsoa, F; De Urturi, DS; Rodríguez-Agudo, R; Lachiondo-Ortega, S; Mercado-Gomez, M; de Juan, ...

MOLECULAR METABOLISM

2021-07-13

LUZP1 Controls Cell Division, Migration and Invasion Through Regulation of the Actin Cytoskeleton

Bozal-Basterra, L; Gonzalez-Santamarta, M; Muratore, V; Martín-Martín, N; Ercilla, A; Rodríguez, JA; Carracedo, A; Sutherland, JD; Barrio, R;

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY

2021-04-01

Virtual meeting, real and sound science: report of the 17th Meeting of the Spanish Society for Developmental Biology (SEBD-2020) Virtual Meeting. 18th to 20th November 2020 .

Araújo, SJ; Almudi, I; Bozal-Basterra, L; Casares, F; Casas-Tintó, S; Escalante, A; Garcia-Moreno, F; Losada-Pérez, M; Maeso, I; Marcon, L; Ocaña, OH; Pampliega, O; Rada-Iglesias, A; Rayon, ...

INTERNATIONAL JOURNAL OF DEVELOPMENTAL BIOLOGY

2021-01-01

Multi-Omics Integration Highlights the Role of Ubiquitination in CCl4-Induced Liver Fibrosis

Mercado-Gómez, M; Lopitz-Otsoa, F; Azkargorta, M; Serrano-Maci, M; Lachiondo-Ortega, S; Goikoetxea-Usandizaga, N; Rodríguez-Agudo, R; Fernández-Ramos, D; Bizkarguenaga, M; Gutiérrez-de Juan, ...

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

2020-12-01

HuR/ELAVL1 drives malignant peripheral nerve sheath tumor growth and metastasis

Palomo-Irigoyen, M; Pérez-Andrés, E; Iruarrizaga-Lejarreta, M; Barreira-Manrique, A; Tamayo-Caro, M; Vila-Vecilla, L; Moreno-Cugnon, L; Beitia, N; Medrano, D; Fernández-Ramos, D; Lozano, JJ; ...

JOURNAL OF CLINICAL INVESTIGATION

2020-07-01

LUZP1, a novel regulator of primary cilia and the actin cytoskeleton, is a contributing factor in Townes-Brocks Syndrome

Bozal-Basterra, L; Gonzalez-Santamarta, M; Muratore, V; Bermejo-Arteagabeitia, A; Da Fonseca, C; Barroso-Gomila, O; Azkargorta, M; Iloro, I; Pampliega, O; Andrade, R; Martín-Martín, N; Branon, ...

ELIFE

2020-06-18