Gauge Field Theory in the Infrared Regime

We propose that the low energy behavior of a pure gauge theory can be studied by simply assuming violation of Lorentz invariance which is implemented through a deformation of the canonical Poisson brackets of the theory depending on an infrared scale. The resulting theory is equivalent to a pure gauge theory with a Chern-Simons like term. It is shown that at low energies this theory can be identified with three dimensional QCD where the mass of the fermion is related to the infrared scale.Comment: 4pp, ReVte

Tuning the program transformers from LCC to PDL

This work proposes an alternative definition of the so-called program transformers used to obtain reduction axioms in the Logic of Communication and Change (LCC). Our proposal uses an elegant matrix treatment of Brzozowski’s equational method instead of Kleene’s translation from finite automata to regular expressions. The two alternatives are shown to be equivalent, with Brzozowski’s method having the advantage of generating smaller expressions for models with average connectivity

Mutations in the EXT1 and EXT2 genes in Spanish patients with multiple osteochondromas

Multiple osteochondromas is an autosomal dominant skeletal disorder characterized by the formation of multiple cartilage-capped tumours. Two causal genes have been identified, EXT1 and EXT2, which account for 65% and 30% of cases, respectively. We have undertaken a mutation analysis of the EXT1 and EXT2 genes in 39 unrelated Spanish patients, most of them with moderate phenotype, and looked for genotype-phenotype correlations. We found the mutant allele in 37 patients, 29 in EXT1 and 8 in EXT2. Five of the EXT1 mutations were deletions identified by MLPA. Two cases of mosaicism were documented. We detected a lower number of exostoses in patients with missense mutation versus other kinds of mutations. In conclusion, we found a mutation in EXT1 or in EXT2 in 95% of the Spanish patients. Eighteen of the mutations were novel.Fil: Sarrión, P.. Universidad de Barcelona; EspañaFil: Sangorrin, A.. Hospital Sant Joan de Déu; EspañaFil: Urreizti, R.. Universidad de Barcelona; EspañaFil: Delgado, María Andrea. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de Córdoba; ArgentinaFil: Artuch, R.. Hospital Sant Joan de Déu; EspañaFil: Martorell, L.. Hospital Sant Joan de Déu; EspañaFil: Armstrong, J.. Hospital Sant Joan de Déu; EspañaFil: Anton, J.. Hospital Sant Joan de Déu; EspañaFil: Torner, F.. Hospital Sant Joan de Déu; EspañaFil: Vilaseca, M. A.. Hospital Sant Joan de Déu; EspañaFil: Nevado, J.. Hospital Universitario La Paz; EspañaFil: Lapunzina, P.. Hospital Universitario La Paz; EspañaFil: Asteggiano, Carla Gabriela. Universidad Nacional de Córdoba; Argentina. Universidad Católica de Córdoba; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Balcells, S.. Universidad de Barcelona; EspañaFil: Grinberg, D.. Universidad de Barcelona; Españ

Noncommutative Wess-Zumino-Witten actions and their Seiberg-Witten invariance

We analyze the noncommutative two-dimensional Wess-Zumino-Witten model and its properties under Seiberg-Witten transformations in the operator formulation. We prove that the model is invariant under such transformations even for the noncritical (non chiral) case, in which the coefficients of the kinetic and Wess-Zumino terms are not related. The pure Wess-Zumino term represents a singular case in which this transformation fails to reach a commutative limit. We also discuss potential implications of this result for bosonization.Comment: Version to appear in Nuclear Physics

A broad spectrum of genomic changes in latinamerican patients with EXT1/EXT2-CDG

Multiple osteochondromatosis (MO), or EXT1/EXT2-CDG, is an autosomal dominant O-linked glycosylation disorder characterized by the formation of multiple cartilage-capped tumors (osteochondromas). In contrast, solitary osteochondroma (SO) is a non-hereditary condition. EXT1 and EXT2, are tumor suppressor genes that encode glycosyltransferases involved in heparan sulfate elongation. We present the clinical and molecular analysis of 33 unrelated Latin American patients (27 MO and 6 SO). Sixty-three percent of all MO cases presented severe phenotype and two malignant transformations to chondrosarcoma (7%). We found the mutant allele in 78% of MO patients. Ten mutations were novel. The disease-causing mutations remained unknown in 22% of the MO patients and in all SO patients. No second mutational hit was detected in the DNA of the secondary chondrosarcoma from a patient who carried a nonsense EXT1 mutation. Neither EXT1 nor EXT2 protein could be detected in this sample. This is the first Latin American research program on EXT1/EXT2-CDG.Fil: Delgado, M. A.. Universidad Nacional de Córdoba. Facultad de Medicina; ArgentinaFil: Martinez Domenech, G.. Universidad Nacional de Córdoba. Facultad de Medicina; ArgentinaFil: Sarrión, P.. Universidad de Barcelona; EspañaFil: Urreizti, R.. Universidad de Barcelona; EspañaFil: Zecchini, L.. Hospital de Niños de la Santísima Trinidad; ArgentinaFil: Robledo, H. H.. Hospital de Niños de la Santísima Trinidad; ArgentinaFil: Segura, F.. Universidad Nacional de Córdoba; ArgentinaFil: Dodelson de Kremer, Raquel. Universidad Nacional de Córdoba. Facultad de Medicina; ArgentinaFil: Balcells, S.. Universidad de Barcelona; EspañaFil: Grinberg, D.. Universidad de Barcelona; EspañaFil: Asteggiano, Carla Gabriela. Universidad Nacional de Córdoba. Facultad de Medicina. Centro de Est.de Las Metabolopatias Congenitas. Cátedra de Clinica Pediatrica; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba; Argentin

Local delivery of optimized nanobodies targeting the PD-1/PD-L1 axis with a self-amplifying RNA viral vector induces potent antitumor responses

Despite the success of immune checkpoint blockade for cancer therapy, many patients do not respond adequately. We aimed to improve this therapy by optimizing both the antibodies and their delivery route, using small monodomain antibodies (nanobodies) delivered locally with a self-amplifying RNA (saRNA) vector based on Semliki Forest virus (SFV). We generated nanobodies against PD-1 and PD-L1 able to inhibit both human and mouse interactions. Incorporation of a dimerization domain reduced PD-1/PD-L1 IC50 by 8- and 40-fold for antiPD-L1 and anti-PD-1 nanobodies, respectively. SFV viral particles expressing dimeric nanobodies showed a potent antitumor response in the MC38 model, resulting in >50% complete regressions, and showed better therapeutic efficacy compared to vectors expressing conventional antibodies. These effects were also observed in the B16 melanoma model. Although a short-term expression of nanobodies was observed due to the cytopathic nature of the saRNA vector, it was enough to generate a strong proinflammatory response in tumors, increasing infiltration of NK and CD8+ T cells. Delivery of the SFV vector expressing dimeric nanobodies by local plasmid electroporation, which could be more easily translated to the clinic, also showed a potent antitumor effect

Metabolic engineering to simultaneously activate anthocyanin and proanthocyanidin biosynthetic pathways in Nicotiana spp

[EN] Proanthocyanidins (PAs), or condensed tannins, are powerful antioxidants that remove harmful free oxygen radicals from cells. To engineer the anthocyanin and proanthocyanidin biosynthetic pathways to de novo produce PAs in two Nicotiana species, we incorporated four transgenes to the plant chassis. We opted to perform a simultaneous transformation of the genes linked in a multigenic construct rather than classical breeding or retransformation approaches. We generated a GoldenBraid 2.0 multigenic construct containing two Antirrhinum majus transcription factors (AmRosea1 and AmDelila) to upregulate the anthocyanin pathway in combination with two Medicago truncatula genes (MtLAR and MtANR) to produce the enzymes that will derivate the biosynthetic pathway to PAs production. Transient and stable transformation of Nicotiana benthamiana and Nicotiana tabacum with the multigenic construct were respectively performed. Transient expression experiments in N. benthamiana showed the activation of the anthocyanin pathway producing a purple color in the agroinfiltrated leaves and also the effective production of 208.5 nmol (-) catechin/g FW and 228.5 nmol (-) epicatechin/g FW measured by the p-dimethylaminocinnamaldehyde (DMACA) method. The integration capacity of the four transgenes, their respective expression levels and their heritability in the second generation were analyzed in stably transformed N. tabacum plants. DMACA and phoroglucinolysis/HPLC-MS analyses corroborated the activation of both pathways and the effective production of PAs in T0 and T1 transgenic tobacco plants up to a maximum of 3.48 mg/g DW. The possible biotechnological applications of the GB2.0 multigenic approach in forage legumes to produce "bloatsafe" plants and to improve the efficiency of conversion of plant protein into animal protein (ruminal protein bypass) are discussed.This work was supported by grants BIO2012-39849-C02-01 and BIO2016-75485-R from the Spanish Ministry of Economy and Competitiveness (MINECO) (http://www.idi.mineco.gob.es/portal/site/MICINN) to LAC and a fellowship of the JAE-CSIC program to SF. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.Fresquet-Corrales, S.; Roque Mesa, EM.; Sarrión-Perdigones, A.; Rochina, M.; López-Gresa, MP.; Díaz-Mula, HM.; Belles Albert, JM.... (2017). Metabolic engineering to simultaneously activate anthocyanin and proanthocyanidin biosynthetic pathways in Nicotiana spp. PLoS ONE. 12(9). https://doi.org/10.1371/journal.pone.0184839Se018483912

Rowing against the wind: how do times of austerity shape academic entrepreneurship in unfriendly environments?

[EN] Academic spin-offs (ASOs) help universities transfer knowledge or technology through business projects developed by academic staff. This investigation aims at analyzing the critical factors for spin-off creation at universities operating in crisis-raven, entrepreneurship-unfriendly environments. Such factors revolve around four types of resources: environmental, institutional, organizational, and personal. Focusing on a Southern European context, as an example of an unfriendly environment affected by economic crisis, an entrepreneurial university (the Technical University of Valencia in Spain, UPV) is our research setting. Through a case study approach, we examine the potential of UPV as a springboard for ASOs. Our results show an adverse local environment, a rather favorable influence of institutional and organizational drivers, and a mixed role of personal factors. Our findings illustrate that UPV consistently supports spin-off creation due to a greater (rather positive) reflexivity from its institutional, organizational and personal resources than the (negative) imprinting of the unfriendly environment. This helps counter-balance the structural unfriendliness for academic entrepreneurship, and trigger a crisis-led risk-taking attitude by academic staff. Hence, UPV should continue with its current strategy of supporting academic entrepreneurship, and might transfer best practices to other universities also affected by unfavorable environmental conditions. Generally speaking, we would advise universities facing adverse circumstances to develop rules and mechanisms for academic entrepreneurship, carefully revise and improve malfunctions, and become involved throughout the whole process of spin-off development. All in all, our study advances understanding of how the different drivers for ASO creation can be revamped by universities located in unfriendly environments, having in mind the key role that universities play in fostering social and economic development through academic entrepreneurship in such environments.The authors would like to thank the Universitat Politecnica de Valencia (grant PAID-06-12-0916), and the Spanish Ministry of Economy and Competitiveness (grant ECO2011-29863), for their financial support for this research.Seguí-Mas, E.; Oltra, V.; Tormo-Carbó, G.; Sarrión Viñes, F. (2017). Rowing against the wind: how do times of austerity shape academic entrepreneurship in unfriendly environments?. International Entrepreneurship and Management Journal. 1-42. doi:10.1007/s11365-017-0478-zS142Acs, Z. J., Audretsch, D. B., & Lehmann, E. E. (2013). The knowledge spillover theory of entrepreneurship. Small Business Economics, 41, 757–774.Alemany, L. (2011). Libro blanco de la iniciativa emprendedora en España. Resource document. ISEAD. http://idl.isead.edu.es:8080/jspui/bitstream/123456789/859/1/658ALElib.pdf . Accessed 31 October 2015.Algieri, B., Aquino, A., & Succurro, M. (2013). Technology transfer offices and academic spin-off creation: the case of Italy. Journal of Technology Transfer, 38(4), 382–400.ARWU (2017). Academic Ranking of World Universities 2017. Resource document. http://www.shanghairanking.com/ARWU2017.html . Accesed 15 August 2017.Ashcroft, B., Holden, D., & Low, K. (2004). Potential entrepreneurs and the self employment choice decision. In Strathclyde Discussion papers in Economics, 4–16. Glasglow: University of Strathclyde.Autio, E., & Kauranen, I. (1994). Technologist-entrepreneurs versus nonentrepreneurial technologists: Analysis of motivational triggering factors. Entrepreneurship & Regional Development, 6, 315–328.Autio, E., Kenney, M., Mustar, P., Siegel, D., & Wright, M. (2014). Entrepreneurial innovation: The importance of context. Research Policy, 43, 1097–1108.Bonnacorsi, A., Colombo, M. G., Guerini, M., & Rossi-Lamastra, C. (2013). University specialization and new firm creation across industries. Small Business Economics, 41, 837–863.Bruneel, J., Van de Velde, E., & Clarysse, B. (2013). Impact of the type of corporate spin-off on growth. Entrepreneurship Theory and Practice, 37, 943–959.CampusHabitat5U (2017). International Campus of Excellence. Resource document. UPV. http://campushabitat5u.es/?lang=en . Accessed 5 October 2017.Chiesa, V., & Piccaluga, A. (2000). Exploitation and diffusion of public research: The chase of academic spin-offs companies in Italy. R&D Management, 30, 329–339.Clark, B. R. (1998). Creating entrepreneurial universities: Organizational pathways of transformation. New York: IAU Press.Clarysse, B., & Moray, N. (2004). A process study of entrepreneurial team formation: The case of research-based spin-off. Journal of Business Venturing, 19, 55–79.Cohen, M., Nelson, R., & Walsh, J. (2002). Links and impacts: The influence of public research on industrial R&D. Management Science, 48, 1–23.Creswell, J.W. & Clark, V. (2011). Designing and Conducting Mixed Methods Research. SAGE Publications.De Cleyn, S. H., Braet, J., & Klofsten, M. (2015). How human capital interacts with the early development of academic spin-offs. International Entrepreneurship and Management Journal, 11(3), 599–621.Doutriaux, J., & Peterman, D. (1982). Technology transfer and academic entrepreneurship. Babson Park: Frontiers of Entrepreneurship Research, Babson College Entrepreneurship Research Conference (BCERC).Eisenhardt, K. M. (1989). Building Theories from Case Study Research. Academy of Management Review, 14(4), 532–550.European Commission (2017). Erasmus 2013–14. Top 500 higher education institutions receiving Erasmus students. Resource document. EC. http://ec.europa.eu/dgs/education_culture/repository/education/library/statistics/2014/erasmus-receiving-institutions_en.pdf Accessed 5 October 2017.Eurovoc (2017). Mutilingual Thesaurus of the European Union. Resource document. http://eurovoc.europa.eu Accessed 03 February 2017.Franzoni, C. & Lissoni, F. (2006). Academic entrepreneurship, patents and spinoffs: Critical issues and lessons for Europe. CESPRI, Università Commerciale “Luigi Bocconi”. Working Paper No. 80.Fritsch, M., & Aamoucke, R. (2013). Regional public research, higher education, and innovative start-ups: An empirical investigation. Small Business Economics, 41, 865–885.Gartner, W. B. (1985). A conceptual framework for describing the phenomenon of new venture creation. The Academy of Management Review, 10, 696–706.Gartner, W. B. (1988). Who is an entrepreneur? is the wrong question. American Journal of Small Business, 12, 11–32.Geuna, A., & Nesta, L. J. J. (2006). University Patenting and its Effects on Academic Research: The merging European Evidence. Research Policy, 35, 790–807.Gibbert, M., & Ruigrok, W. (2010). The “What” and “How” of the case Study Rigor: Three Strategies based on Published Work. Organizational Research Methods, 13(4), 710–737.Gómez Gras, J. M., Galiana Lapera, D. R., Mira Solves, I., Verdú Jover, A. J., & Sancho Azuar, J. (2008). An empirical approach to the organisational determinants of spin-off creation in European universities. International Entrepreneurship and Management Journal, 4(2), 187–198.Grandi, A., & Grimaldi, R. (2005). Academics' organizational characteristics and the generation of successful business ideas. Journal of Business Venturing, 20(6), 821–845.Güemes, J.J. (2011), “Global Entrepreneurship Monitor. Informe GEM España 2010”. Resource document. GEM España. http://www.gemconsortium.org/docs/download/616. Accessed 15 January 2015 .Guerrero, M., & Urbano, D. (2012). The development of an entrepreneurial university. Journal of Technology Transfer, 37(1), 43–74.Guerrero, M., Urbano, D., Cunningham, J., & Organ, D. (2014). Entrepreneurial universities in two European regions: a case study comparison. Journal of Technology Transfer, 39(3), 415–434.Hoang, H., & Antoncic, B. (2003). Network-based research in entrepreneurship: A critical review. Journal of Business Venturing, 18(2), 165–187.Hofstede, G. (1980). Culture’s Consequences. International differences in work-related values. Beverly Hills: Sage.Hofstede, G. (2001). Culture’s consequences: Comparing values, behaviours, institutions, and organizations across nations (2nd ed.). Thousand Oaks: Sage.Hülsbeck, M., & Pickavé, E. N. (2014). Regional knowledge production as determinant of high-technology entrepreneurship: Empirical evidence for Germany. International Entrepreneurship and Management Journal, 10, 121–138.INE (2016). INEbase: Operaciones estadísticas. Instituto Nacional de Estadística (National [Spanish] Statistical Institute). Resource document. INE. http://www.ine.es/inebmenu/indice.htm . Accessed 2 July 2016.Kalar, B., & Antoncic, B. (2015). The entrepreneurial university, academic activities and technology and knowledge transfer in four European countries. Technovation, 36-37, 1–11.Kroll, H. (2009). Demonstrating the instrumentality of motivation oriented approaches for the explanation of academic spin-off formation—an application based on the Chinese case. International Entrepreneurship and Management Journal, 5, 97–116.LAEI (2013). Ley 14/2013, de 27 de septiembre, de Apoyo a Emprendedores y su Internacionalización (‘Act of Support to Entrepreneurs and their Internationalization’). Government of Spain, 27 September. Resource document: http://www.boe.es/boe/dias/2013/09/28/pdfs/BOE-A-2013-10074.pdf . Accessed 10 March 2016.Lam, A., & De Campos, A. (2015). Content to be sad’ or ‘runaway apprentice’? The psychological contract and career agency of young scientists in the entrepreneurial university. Human Relations, 68(5), 811–841.LCTI (2011). Ley 14/2011, de 1 de junio, de la Ciencia, la Tecnología y la Innovación (‘Science, Technology and Innovation Act’). Government of Spain, 1 June. Resource document: http://www.boe.es/boe/dias/2011/06/02/pdfs/BOE-A-2011-9617.pdf . Accessed 10 March 2016.León-Darder, F. (2016). La internacionalització de l’empresa valenciana. In E. Seguí-Mas (Ed.), Una nova via per a l’empresa valenciana (pp. 61–80). Catarroja: Editorial Afers & Fundació Nexe.LES (2011). Ley 2/2011, de 4 de marzo, de Economía Sostenible (‘Sustainable Economy Act’). Government of Spain, 4 March, Resource document. http://www.boe.es/boe/dias/2011/03/05/pdfs/BOE-A-2011-4117.pdf. Accessed 10 March 2016 .Leyden, D. P., & Link, A. N. (2013). Knowledge spillovers, collective entrepreneurship, and economic growth: The role of universities. Small Business Economics, 41, 797–817.Lindelöf, P., & Löfsten, H. (2006). Environmental hostility and firm behavior – An empirical examination of new technology-based firms on science parks. Journal of Small Business Management, 44(3), 386–406.Link, N., & Scott, T. (2005). Opening the ivory’s tower door: An analysis of the determinants of the formation of US university spin-off companies. Research Policy, 34, 1106–1112.Lockett, A., & Wright, M. (2005). Resources, capabilities, risk capital and the creation of university spin-out companies. Research Policy, 34, 1043–1057.LOMLOU (2007). Ley Orgánica 4/2007, de 12 de abril, por la que se modifica la Ley Orgánica 6/2011, de 21 de diciembre, de Universidades (‘Act of Modification of the University Act’). Government of Spain, 12 April. Resource document. https://www.boe.es/boe/dias/2007/04/13/pdfs/A16241-16260.pdf (accessed 11 March 2016).LOU (2001). Ley Orgánica 6/2001, de Universidades (‘University Act’). Government of Spain, 21 December. Resource document: https://www.boe.es/boe/dias/2001/12/24/pdfs/A49400-49425.pdf . Accessed 11 March 2016.Martinelli, A., Meyer, M., & Von Tunzelmann, N. (2008). Becoming an entrepreneurial university? A case study of knowledge exchange relationships and faculty attitudes in a medium-sized, research-oriented university. Journal of Technology Transfer, 33, 259–283.Martínez Carrascal, C. & Mulino Ríos, M. (2014). La evolución del crédito bancario a las empresas españolas según su tamaño. Un análisis basado en la explotación conjunta de la información de la CIR y de la CBI, Boletín Económico - Banco de España, Enero (January), pp. 117–125.Mathias, B. D., Williams, D. W., & Smith, A. R. (2015). Entrepreneurial inception: The role of imprinting in entrepreneurial action. Journal of Business Venturing, 30(1), 11–28.MIET (Spanish Ministry of Industry, Energy and Tourism) (2012). Estadísticas Pyme. Evolución e indicadores. No. 10″, Resource document. http://www.ipyme.org/Publicaciones/ESTADISTICAS_PYME_N10_2011.pdf. Accessed 2 May 2016 .Miles, M.B. & Huberman, A.M. (2008). Qualitative Data Analysis: an expanded sourcebook. Sage Publications.Morales-Gualdrón, S. Y., Gutiérrez-Gracias, & Roig Dobón, S. (2009). The entrepreneurial motivation in academia: A multidimensional construct. International Entrepreneurship and Management Journal, 6, 301–317.Mosey, S., & Wright, M. (2007). From human capital to social capital: A longitudinal study of technology-based academic entrepreneurs. Entrepreneur, 31, 909–936.Mosey, S., Lockett, A., & Westhead, P. (2006). Creating network bridges for university technology transfer: The Medici fellowship programme. Technology Analysis and Strategic Management, 18, 71–91.Mosey, S., Wright, M., & Clarysse, B. (2012a). Transforming traditional university structures for the knowledge economy through multidisciplinary institutes. Cambridge Journal of Economics, 36, 587–607.Mosey, S., Noke, H., & Binks, M. (2012b). The influence of human and social capital upon the entrepreneurial intentions and destinations of academics. Technology Analysis and Strategic Management, 24, 893–910.Moutinho, R., Au-Yong-Oliveira, M., Coelho, A., & Manso, J. P. (2016). Determinants of knowledge-based entrepreneurship: an exploratory approach. International Entrepreneurship and Management Journal, 12(1), 171–197.Mowery, D. C., Nelson, R. R., Sampat, B. N., & Ziedonis, A. A. (2001a). The growth of patenting and licensing by US universities: an assessment of the effects of Bayle-Dole Act of 1980. Research Policy, 30(1), 99–119.Mowery, D. C., Sampat, B. N., & Ziedonis, A. A. (2001b). Learning to patent: institutional experience, learning, and the characyeristics of US university Patents after the Bayle-Dole Act, 1981-1992. Management Science, 48(1), 73–89.O’Shea, R., Allen, J., Chevalier, A., & Roche, F. (2005). Entrepreneurial orientation, technology transfer and spinoff performance of US universities. Research Policy, 34, 994–1009.O’Shea, R., Allen, T., Morse, K., O’Gorman, C., & Roche, F. (2007). Delineating the anatomy of an entrepreneurial university: the Massachusetts Institute of Technology Experience. R&D Management, 37(1), 1–16.O’Shea, R., Chugh, H., & Allen, T. (2008). Determinants and consequences of university spinoff activity: A conceptual framework. Journal of Technology Transfer, 33, 653–666.Ortín, P., Salas, V., Trujillo, M.V., & Vendrell, F. (2007). El spin-off universitario en España como modelo de creación de empresas intensivas en tecnología. Ministerio de Industria, Turismo y Comercio. Secretaría General de Industria. Dirección General de Política de la Pyme. Resource document. http://www.ipyme.org/Publicaciones/Informe spinnoff.pdf . Accessed 2 October 2016.Papaoikonomou, E., Segarra, P., & Li, X. (2012). Entrepreneurship in the context of crisis: Identifying barriers and proposing strategies. International Advances in Economic Research, 18, 111–119.Piperopoulos, P., & Piperopoulos, G. (2010). Is Greece finally on the right path toward entrepreneurship, innovation, and business clusters? International Journal of Public Administration, 33(1), 55–59.Powers, B., & McDougall, P. (2005). University startup formation and technology licensing with firms that go public: A resource-based view of academic entrepreneurship. Journal of Business Venturing, 20, 291–311.Red OTRI (2016). Informe de la Encuesta de Investigación y Transferencia 2014 de las universidades españolas. Resource document. http://www.redotriuniversidades.net/index.php/informa-encuesta/6-encuesta-redotri/informa-encuesta-2014/download . Accessed 22 June 2016.Redero San-Román, M. (2002). Origen y desarrollo de la universidad franquista. Studia Zamorensia, 6, 337–352.Rodríguez-Gulías, M. J., Rodeiro-Pazos, D., & Fernández-López, S. (2017). The effect of university and regional knowledge spillovers on firms’ performance: an analysis of the Spanish USOs. International Entrepreneurship and Management Journal, 13(1), 191–209.Rodríguez-San Pedro, L.E. (2014). Las universidades españolas en su contexto historic. Resource document. Universia. http://universidades.universia.es/universidades-de-pais/historia-de-universidades/historia-universidad-espanola/pasado-reciente/pasado-reciente-multiplicidad-regimen-autonomico.html . Accessed 28 July 2015.Samsom, K., & Gurdon, M. (1990). Entrepreneurial scientist: Organizational performance in scientist-started high technology firms. Forest Park: Frontiers of Entrepreneurship Research, Babson College Entrepreneurship Research Conference (BCERC).Schmitz, A., Urbano, D., Dandolini, G. A., de Souza, J. A., & Guerrero, M. (2017). Innovation and entrepreneurship in the academic setting: A systematic literature review. International Entrepreneurship and Management Journal, 13(2), 369–395.Shane, S., & Khurana, R. (2003). Bringing individuals back in: The effects of career experience on new firm founding. Industrial and Corporate Change, 12, 519–543.Shapero, A., & Sokol, L. (1982). The social dimensions of entrepreneurship. In C. A. Kent, D. L. Sexton, & K. H. Vesper (Eds.), Encyclopaedia of entrepreneurship (pp. 72–90). Englewood Cliffs: Prentice Hall.Smilor, R. W., Gibson, D. V., & Dietrich, G. B. (1990). University spin-out companies: technology start-ups from UT-Austin. Journal of Business Venturing, 5(1), 63–76.Soler i Marco, V. (2009). Creixement i canvi estructural. In V. Soler (Ed.), Economia espanyola i del País Valencià. Valencia: Publicacions de la Universitat de València.Suddaby, R., Bruton, G. D., & Si, S. X. (2015). Entrepreneurship through a qualitative lens: Insights on the construction and/or discovery of entrepreneurial opportunity. Journal of Business Venturing, 30(1), 1–10.Tech Transfer UPV FCR (2016). Air Nostrum, Caixa Popular e IVI entran en el fondo de la UPV. Resource document. TTUPV FCR. http://www.techtransferupv.com/noticias/air-nostrum-caixa-popular-e-ivi-entran-en-el-fondo-de-la-upv/ (4 April) Accessed 10 July 2016.The Times Higher Education (2017). 100 Under 50 Ranking 2017. Resource document. THE. https://www.timeshighereducation.com/world-university-rankings/2017/young-university-rankings#!/page/0/length/-1/sort_by/rank/sort_order/asc/cols/stats . Accessed 15 august 2017.UPV (2007). Instituto IDEAS 15 aniversario (1992–2007). Resource document. UPV. http://www.upv.es/entidades/IDEAS/menu_urlv.html?http://www.upv.es/entidades/IDEAS/info/memoria15a%F1os.pdf . Accessed 10 April 2016.UPV (2011). Corporación empresarial. Resource document. UPV. http://www.upv.es/noticias-upv/noticia-4904-corporacion-emp-es.html . Accessed 10 April 2016.UPV (2014). Plan de emprendimiento global. Resource document. UPV. https://www.upv.es/noticias-upv/noticia-6846-plan-de-emprend-es.html . Accessed 10 April 2016.UPV (2015). Jornadas de Puertas Abiertas 2015–16. Resource document. UPV. www.upv.es/contenidos/ORIENTA/info/jpa_ciclos_2015-16.ppt . Accessed 10 April 2016.UPV (2017a). Spin-Off UPV. Resource document. UPV. http://www.upv.es/entidades/I2T/info/891434normalc.html . Accessed 5 October 2017.UPV (2017b). Ciudad Politécnica de la Innovación. Parque Científico en Red de la Universidad Politécnica de Valencia. Quienes Somos. Presentación. Resource document. UPV. http://cpi.upv.es/quienes-somos/presentacion . Accessed 5 October 2017.UPV (2017c). Servicio de Promoción y Apoyo a la Investigación, la Innovación y la Transferencia. Presentación. Resource document. UPV. http://i2t.webs.upv.es/i2t/presentacion.php. Accessed 5 October 2017 .UPV. (2017d). Tech Transfer UPV. UPV: Resource document http://www.upv.es/noticias-upv/noticia-8355-tech-transfer-u-es.html. Accessed 5 October 2017 .UPV (2017e). Mission statement, vision and values. Resource document. UPV. https://www.upv.es/organizacion/la-institucion/misionvisionvalores-plan-upv-en.html Accessed 17 October 2017.Vargas Vasserot, C. (2012). Las spin-offs académicas y su posible configuración como empresas de economía social. REVESCO. Revista de Estudios Cooperativos, 107, 186–205.VLC/Campus (2017). VLC/Campus. Valencia, International Campus of Excellence. Resource document. UPV. http://www.vlc-campus.com/en . Accessed 5 October 2017.Walter, A., Auer, M., & Ritter, T. (2006). The impact of network capabilities and entrepreneurial orientation on university spin-off performance. Journal of Business Venturing, 21(4), 541–567.Weatherston, J. (1995). Academic Entrepreneurs: Is a spin-off Company too risky. Proceedings of the 40th International Council on Small Business, Sydney, 18–21.Willoughby, M., Talon, J., Millet, J., & Ayats, C. (2013). University services for fostering creativity in hi-tech firms. The Service Industries Journal, 33, 1103–1116.Wright, M., & Mosey, S. (2012). Strategic entrepreneurship, resource orchestration and growing spin-offs from universities. Technology Analysis and Strategic Management, 24, 911–927.Wright, M., Clarysse, B., Mustar, P., & Lockett, A. (2007). Academic Entrepreneurship in Europe. Cheltenham: Edward Elgar.Yin, R. K. (1994). Case study research: Design and methods (2nd ed.). Sage: Thousand Oaks.Yusof, M., & Jain, K. J. (2010). Categories of university-level entrepreneurship: A literature survey. International Entrepreneurship and Management Journal, 6(1), 81–86

MUTATIONS IN THE EXT1 AND EXT2 GENES IN SPANISH PATIENTS WITH MULTIPLE OSTEOCHONDROMAS

Multiple osteochondromas is an autosomal dominant skeletal disorder characterized by the  formation of multiple cartilage-capped tumours. Two causal genes have been identified, EXT1 and EXT2, which account for 65% and 30% of cases, respectively. We have undertaken a mutation analysis of the EXT1 and EXT2 genes in 39 unrelated Spanish patients, most of them with moderate phenotype, and looked for genotype-phenotype correlations.Wefound the mutant allele in 37 patients, 29 inEXT1 and 8 inEXT2. Five of the EXT1 mutations were deletions identified by MLPA. Two cases of mosaicism were documented. We detected a lower number of exostoses in patients with missense mutation versus other kinds of mutations. In conclusion, we found a mutation in EXT1 or in EXT2 in 95% of the Spanish patients. Eighteen of the mutations were novel.publishedVersionOtras Ciencias de la Salu