• The 14-3-3 proteins in regulation of cellular metabolism 

      Kleppe, Rune; Martinez, Aurora; Døskeland, Stein Ove; Haavik, Jan (Peer reviewed; Journal article, 2011-09)
      Thirty years ago, it was discovered that 14-3-3 proteins could activate enzymes involved in amino acid metabolism. In the following decades, 14-3-3s have been shown to be involved in many different signaling pathways that ...
    • Arc is a flexible modular protein capable of reversible self-oligomerization 

      Myrum, Craig; Baumann, Anne; Bustad, Helene J.; Flydal, Marte Innselset; Mariaule, Vincent; Alvira, Sara; Cuéllar, Jorge; Haavik, Jan; Soulé, Jonathan; Valpuesta, José María; Márquez, José Antonio; Martinez, Aurora; Bramham, Clive R. (Peer reviewed; Journal article, 2015)
      The immediate early gene product Arc (activity-regulated cytoskeleton-associated protein) is posited as a master regulator of long-term synaptic plasticity and memory. However, the physicochemical and structural properties ...
    • The Benefit of Large Neutral Amino Acid Supplementation to a Liberalized Phenylalanine-Restricted Diet in Adult Phenylketonuria Patients: Evidence from Adult Pah-Enu2 Mice 

      Van Vliet, Danique; van der Goot, Els; van Ginkel, Wiggert G; van Faassen, Martijn H J R; de Blaauw, Pim; Kema, Ido P.; Martinez, Aurora; Heiner-Fokkema, M Rebecca; van der Zee, Eddy A; van Spronsen, Francjan J. (Peer reviewed; Journal article, 2019-09-19)
      Many phenylketonuria (PKU) patients cannot adhere to the severe dietary restrictions as advised by the European PKU guidelines, which can be accompanied by aggravated neuropsychological impairments that, at least in part, ...
    • Conformational Sampling and Nucleotide-Dependent Transitions of the GroEL Subunit Probed by Unbiased Molecular Dynamics Simulations 

      Skjærven, Lars; Grant, Barry; Muga, Arturo; Teigen, Knut; McCammon, J. Andrew; Reuter, Nathalie; Martinez, Aurora (Peer reviewed; Journal article, 2011-03-10)
      GroEL is an ATP dependent molecular chaperone that promotes the folding of a large number of substrate proteins in E. coli. Large-scale conformational transitions occurring during the reaction cycle have been characterized ...
    • Conformational stability and activity analysis of two hydroxymethylbilane synthase mutants, K132N and V215E, with different phenotypic association with acute intermittent porphyria 

      Bustad, Helene J.; Vorland, Marta; Rønneseth, Eva; Sandberg, Sverre; Martinez, Aurora; Toska, Karen (Peer reviewed; Journal article, 2013-08)
      The autosomal dominantly inherited disease AIP (acute intermittent porphyria) is caused by mutations in HMBS [hydroxymethylbilane synthase; also known as PBG (porphobilinogen) deaminase], the third enzyme in the haem ...
    • Conformational stabilization as a strategy to prevent nucleophosmin mislocalization in leukemia 

      Urbaneja, María A.; Skjærven, Lars; Aubi Catevilla, Oscar; Underhaug, Jarl; López, David J.; Arregi, Igor; Alonso-Mariño, Marián; Cuevas, Andoni; Rodríguez, José A.; Martinez, Aurora; Bañuelos, Sonia (Peer reviewed; Journal article, 2017-10-24)
      Nucleophosmin (NPM) is a nucleolar protein involved in ribosome assembly and cell homeostasis. Mutations in the C-terminal domain of NPM that impair native folding and localization are associated with acute myeloid leukemia ...
    • Cuticle integrity and biogenic amine synthesis in Caenorhabditis elegans require the cofactor tetrahydrobiopterin (BH4) 

      Loer, Curtis M.; Calvo, Ana C; Watschinger, Katrin; Werner-Felmayer, Gabriele; O'Rourke, Delia; Stroud, Dave; Tong, Amy; Gotenstein, Jennifer R.; Chisholm, Andrew D.; Hodgkin, Jonathan; Werner, Ernst R.; Martinez, Aurora (Peer reviewed; Journal article, 2015-03-24)
      Tetrahydrobiopterin (BH4) is the natural cofactor of several enzymes widely distributed among eukaryotes, including aromatic amino acid hydroxylases (AAAHs), nitric oxide synthases (NOSs), and alkylglycerol monooxygenase ...
    • Effect of nerve injury on the number of dorsal root ganglion neurons and autotomy behavior in adult Bax-deficient mice 

      Lyu, Chuang; Lyu, Gong-Wei; Martinez, Aurora; Shi, Tie-Jun (Peer reviewed; Journal article, 2017-08-30)
      Background: The proapoptotic molecule BAX, plays an important role in mitochondrial apoptotic pathway. Dorsal root ganglion (DRG) neurons depend on neurotrophic factors for survival at early developmental stages. Withdrawal ...
    • Effective polyethylene glycol passivation for the inhibition of surface interactions of peripheral blood mononuclear cells and platelets 

      Sauter, Alexander; Richter, Gunther; Micoulet, Alexandre; Martinez, Aurora; Spatz, Joachim P.; Appel, Silke (Peer reviewed; Journal article, 2013-06-20)
      The inhibition of unspecific adhesion of human white blood cells is a prerequisite for applications requiring the control of defined surface interactions. In this study, a passivation agent based on polyethylene glycol ...
    • EU-OPENSCREEN: A novel collaborative approach to facilitate chemical biology 

      Brennecke, Philip; Rasina, Dace; Aubi Catevilla, Oscar; Herzog, Katja; Landskron, Johannes; Cautain, Bastien; Vicente, Francisca; Quintana, Jordi; Mestres, Jordi; Stechmann, Bahne; Ellinger, Bernhard; Brea, Jose; Kolanowski, Jacek L.; Pilarski, Radosław; Orzáez, Mar; Pineda-Lucena, Antonio; Laraia, Luca; Nami, Faranak; Zielenkiewicz, Piotr; Paruch, Kamil; Hansen, Espen; von Kries, Jens P.; Neuenschwander, Martin; Specker, Edgar; Bartunek, Petr; Šímová, Šárka; Leśnikowski, Zbigniew; Krauss, Stefan; Lehtiö, Lari; Bilitewski, Ursula; Brönstrup, Mark; Tasken, Kjetil; Jirgensons, Aigars; Lickert, Heiko; Clausen, Mads H.; Andersen, Jeanette hammer; Vicent, Maria J.; Genilloud, Olga; Martinez, Aurora; Nazaré, Marc; Fecke, Wolfgang; Gribbon, Philip (Peer reviewed; Journal article, 2019)
      Compound screening in biological assays and subsequent optimization of hits is indispensable for the development of new molecular research tools and drug candidates. To facilitate such discoveries, the European Research ...
    • Functional studies of tyrosine hydroxylase missense variants reveal distinct patterns of molecular defects in dopa-responsive dystonia 

      Fossbakk, Agnete; Kleppe, Rune; Knappskog, Per; Martinez, Aurora; Haavik, Jan (Peer reviewed; Journal article, 2014-06-03)
      Congenital tyrosine hydroxylase deficiency (THD) is found in autosomal-recessive Dopa-responsive dystonia and related neurological syndromes. The clinical manifestations of THD are variable, ranging from early-onset lethal ...
    • HAMLET forms annular oligomers when deposited with phoshpolipid monolayers 

      Baumann, Anne; Gjerde, Anja Underhaug; Ying, Ming; Svanborg, Catharina; Holmsen, Holm A.; Glomm, Wilhelm; Martinez, Aurora; Halskau, Øyvind (Peer reviewed; Journal article, 2012-04)
      Recently, the anticancer activity of human α-lactalbumin made lethal to tumor cells (HAMLET) has been linked to its increased membrane affinity in vitro, at neutral pH, and ability to cause leakage relative to the inactive ...
    • HAMLET interacts with lipid membranes and perturbs their structure and integrity 

      Mossberg, Ann-Kristin; Puchades, Maja; Halskau, Øyvind; Baumann, Anne; Lanekoff, Ingela; Chao, Yinxia; Martinez, Aurora; Svanborg, Catharina; Karlsson, Roger (Peer reviewed; Journal article, 2010-01-23)
      Background Cell membrane interactions rely on lipid bilayer constituents and molecules inserted within the membrane, including specific receptors. HAMLET (human α-lactalbumin made lethal to tumor cells) is a tumoricidal ...
    • Modelling cellular signal communication mediated by phosphorylation dependent interaction with 14-3-3 proteins 

      Kleppe, Rune; Ghorbani, Sadaf; Martinez, Aurora; Haavik, Jan (Peer reviewed; Journal article, 2014-01-03)
      The 14-3-3 proteins are important effectors of Ser/Thr phosphorylation in eukaryotic cells. Using mathematical modelling we investigated the roles of these proteins as effectors in signalling pathways that involve ...
    • The N-terminal sequence of tyrosine hydroxylase is a conformationally versatile motif that binds 14-3-3 proteins and membranes 

      Skjevik, Åge Aleksander; Mileni, Mauro; Baumann, Anne; Halskau, Øyvind; Teigen, Knut; Raymond C., Stevens; Martinez, Aurora (Peer reviewed; Journal article, 2014-01-09)
      Tyrosine hydroxylase (TH) catalyzes the rate-limiting step in the synthesis of catecholamine neurotransmitters, and a reduction in TH activity is associated with several neurological diseases. Human TH is regulated, among ...
    • The Peripheral Binding of 14-3-3γ to Membranes Involves Isoform-Specific Histidine Residues 

      Bustad, Helene J.; Skjærven, Lars; Ying, Ming; Halskau, Øyvind; Baumann, Anne; Rodriguez-Larrea, David; Costas, Miguel; Underhaug, Jarl; Sanchez-Ruiz, Jose M.; Martinez, Aurora (Peer reviewed; Journal article, 2012-11-26)
      Mammalian 14-3-3 protein scaffolds include seven conserved isoforms that bind numerous phosphorylated protein partners and regulate many cellular processes. Some 14-3-3-isoforms, notably γ, have elevated affinity for ...
    • A Pharmacological Chaperone Therapy for Acute Intermittent Porphyria 

      Bustad, Helene J.; Toska, Karen; Schmitt, Caroline; Vorland, Marta; Skjærven, Lars; Kallio, Juha Pekka; Simonin, Sylvie; Lettéron, Philippe; Underhaug, Jarl; Sandberg, Sverre; Martinez, Aurora (Peer reviewed; Journal article, 2019-12-03)
      Mutations in hydroxymethylbilane synthase (HMBS) cause acute intermittent porphyria (AIP), an autosomal dominant disease where typically only one HMBS allele is mutated. In AIP, the accumulation of porphyrin precursors ...
    • Phenylalanine Hydroxylase from Legionella pneumophila Is a Thermostable Enzyme with a Major Functional Role in Pyomelanin Synthesis 

      Flydal, Marte Innselset; Chatfield, Christa H.; Zheng, Huaixin; Gunderson, Felizza F.; Aubi, Oscar; Cianciotto, Nicholas P.; Martinez, Aurora (Peer reviewed; Journal article, 2012-09-26)
      Background: Legionella pneumophila is a pathogenic bacterium that can cause Legionnaires’ disease and other non-pneumonic infections in humans. This bacterium produces a pyomelanin pigment, a potential virulence factor ...
    • Phosphorylation at serine 31 targets tyrosine hydroxylase tovesicles for transport along microtubules 

      Jorge Finnigan, Ana; Kleppe, Rune; K C, Kunwar Jung; Ying, Ming; Marie, Michael Bruno Eric; Rios Mondragon, Ivan; Salvatore, Michael F; Saraste, Jaakko; Martinez, Aurora (Peer reviewed; Journal article, 2017)
      Tyrosine hydroxylase (TH) catalyzes the conversion of L-tyrosine into L-DOPA, which is the rate-limiting step in the synthesis of catecholamines, such as dopamine, in dopaminergergic neurons. Low dopamine levels and death ...
    • The Regulatory Subunit of PKA-I Remains Partially Structured and Undergoes b-Aggregation upon Thermal Denaturation 

      Dao, Khanh K.; Pey, Angel; Gjerde, Anja Underhaug; Teigen, Knut; Byeon, In-Ja L.; Døskeland, Stein Ove; Gronenborn, Angela M.; Martinez, Aurora (Peer reviewed; Journal article, 2011-03-04)
      Background: The regulatory subunit (R) of cAMP-dependent protein kinase (PKA) is a modular flexible protein that responds with large conformational changes to the binding of the effector cAMP. Considering its highly dynamic ...