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.
VISIT OUR LAB WEBPAGE for more information
-
Principal Investigator
Rosa Barrio
-
Associate Principal Investigator
James D Sutherland
POSTDOCTORAL RESEARCHER -
Orhi Barroso Gomila
TECHNICIANS / DOCTORAL CANDIDATES -
Mónica Pozo Rodríguez
-
Verónica Muratore
-
Laura Merino Cacho
-
Claudia Guinea Perez
Members
Latest Publications
In memoriam: Huib Ovaa, the brain behind the formula (18/12/1973-19/05/2020)
Barrio, R;Rodriguez, MS
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
2022-12-01
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
2022-12-01
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
2022-06-14
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;Perez, C;Azkargorta, M;Iloro, I;Carracedo, A;Aransay, AM;Elortza, F;Mayor, U;Vertegaal, ACO;Barrio, R;Sutherland, ...
NATURE COMMUNICATIONS
2021-11-18
Neddylation inhibition ameliorates steatosis in NAFLD by boosting hepatic fatty acid oxidation via the DEPTOR-mTOR axis
Serrano-Macia, M;Simon, J;Gonzalez-Rellan, MJ;Azkargorta, M;Goikoetxea-Usandizaga, N;Lopitz-Otsoa, F;De Urturi, DS;Rodriguez-Agudo, R;Lachiondo-Ortega, S;Mercado-Gomez, M;de Juan, VG;Bizkarguenaga, ...
MOLECULAR METABOLISM
2021-11-01
SALL1 Modulates CBX4 Stability, Nuclear Bodies, and Regulation of Target Genes
Giordano, I;Pirone, L;Muratore, V;Landaluze, E;Perez, C;Lang, V;Garde-Lapido, E;Gonzalez-Lopez, M;Barroso-Gomila, O;Vertegaal, ACO;Aransay, AM;Rodriguez, JA;Rodriguez, MS;Sutherland, JD;Barrio, ...
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
2021-09-21
LUZP1 Controls Cell Division, Migration and Invasion Through Regulation of the Actin Cytoskeleton
Bozal-Basterra, L;Gonzalez-Santamarta, M;Muratore, V;Martin-Martin, N;Ercilla, A;Rodriguez, JA;Carracedo, A;Sutherland, JD;Barrio, R
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
2021-04-01