Orhi Barroso Gomila
Orhi Barroso Gomila
Ubiquitin-likes And Development Lab
Address: Bizkaia Science and Technology Park, building 801A, Derio (Bizkaia)

Post-translational modifications by ubiquitin-like modifiers (proteins similar to ubiquitin) can influence many aspects of protein homeostasis, such are stability, localization or activity. SUMOylation is a reversible process by which SUMO, the Small Ubiquitin-like Modifier, is attached to target proteins and modify their properties. We discovered that SUMO has an important role in cholesterol intake, contributing to steroid hormone synthesis through the regulation of the Scavenger Receptors SR-B1 and the nuclear receptor Ftz-f1, homologous to the human Steroidogenic Factor 1, SF-1. The regulation of hormonal synthesis is a crucial step that determines animal viability and size. This novel function of SUMOylation is conserved in several tissues in different organisms. To investigate more in detail the function of the ubiquitin-likes, we are developing new molecular tools to isolate and identify proteins modified by SUMO and other Ubiquitin-like modifiers in human cells and in model systems, such is Drosophila. We generated a versatile, highly specific, easy-to-use toolbox composed of vectors useful to analyze the modification of a protein of interest or to identify modified sub-proteomes. Among the transcription factors regulated by SUMOylation, we are interested on the SPALT-Like (SALL) family of proteins, necessary for numerous biological processes. Mutations in SALL1 and SALL4 cause the rare diseases Townes-Brocks and Okihiro (Duane-Radial Ray) Syndromes, respectively, being also involved in the susceptibility to tumors. Patients might present dysplastic kidneys, supernumerary thumbs, malformed ears, sensorineural hearing loss and severe growth retardation. We are investigating the mechanism by which a truncated form of SALL1 causes the Townes-Brocks symptoms. We hypothesized that these are caused by the malfunctioning of primary cilia and we are currently performing the experiments necessary to prove this hypothesis. Primary fibroblasts derived from a Townes-Brocks patient, which exhibit mutation in the gene SALL1. The fibroblasts were kindly provided by Dr. Wilkie, Oxford, UK. Markers: Acetylated tubulin (red), F-actin (green), DNA (blue). Representation of SUMO evolution across species, with associated functional consequences in human, Blattella and Drosophila.

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Latest Publications

Regulation of the Ebola Virus VP24 Protein by SUMO

Vidal, S;El Motiam, A;Seoane, R;Preitakaite, V;Bouzaher, YH;Gomez-Medina, S;San Martin, C;Rodriguez, D;Rejas, MT;Baz-Martinez, M;Barrio, R;Sutherland, JD;Rodriguez, MS;Munoz-Fontela, C;Rivas, ...

Journal of virology


Targeting PML in triple negative breast cancer elicits growth suppression and senescence.

Arreal, Leire; Piva, Marco; Fernandez, Sonia; Revandkar, Ajinkya; Schaub-Clerigue, Ariane; Villanueva, Josep; Zabala-Letona, Amaia; Pujana, Mikel; Astobiza, Ianire; Cortazar, Ana Rosa; Hermanova, ...

Cell death and differentiation


Impaired proteostasis in rare neurological diseases

Osinalde, N;Duarri, A;Ramirez, J;Barrio, R;de Nanclares, GP;Mayor, U

Seminars in cell & developmental biology


Proteostasis: The network behind the networks

Barrio, R;Sutherland, JD



Using Ubiquitin Binders to Desipher the Ubiquitin Code

Mattern, M;Sutherland, J;Kadimisetty, K;Barrio, R;Rodriguez, MS



PROTEOSTASIS: A European Network to Break Barriers and Integrate Science on Protein Homeostasis

Dissmeyer, N;Coux, O;Rodriguez, MS;Barrio, R



Integrative analysis of transcriptomics and clinical data uncovers the tumor- suppressive activity of MITF in prostate cancer

Valcarcel-Jimenez, L(Valcarcel-Jimenez, Lorea);Macchia, A(Macchia, Alice);Martin-Martin, N(Martin-Martin, Natalia);Cortazar, AR(Rosa Cortazar, Ana);Schaub-Clerigu, A(Schaub-Clerigu, Ariane);Pujana-Vaquerizo, ...

Cell death & disease


Cluster Locator, online analysis and visualization of gene clustering

Obregon, FP(Obregon, Flavio Pazos);Soto, P(Soto, Pablo);Lavin, JL(Lavin, Jose Luis);Cortazar, AR(Cortazar, Ana Rosa);Barrio, R(Barrio, Rosa);Aransay, AM(Aransay, Ana Maria);Cantera, R(Cantera, ...

Bioinformatics (Oxford, England)


Putting the Stress on UFM1 (Ubiquitin-Fold Modifier 1) Protein Ufmylation as a Novel Regulator of Endoplasmic Reticulum Stress in Cardiac Health and Disease

Sutherland, JD(Sutherland, James D.);Barrio, R(Barrio, Rosa)



Drosophila melanogaster White Mutant w(1118) Undergo Retinal Degeneration

Ferreiro, MJ(Jose Ferreiro, Maria);Perez, C(Perez, Coralia);Marchesano, M(Marchesano, Mariana);Ruiz, S(Ruiz, Santiago);Caputi, A(Caputi, Angel);Aguilera, P(Aguilera, Pedro);Barrio, R(Barrio, ...

Frontiers in neuroscience