Roberto Fernández‐Torrón, Mikel García-Puga, José-Ignacio Emparanza, Miren Maneiro, Ana-María Cobo, Juan-José Poza, Juan-Bautista Espinal, Miren Zulaica, Irune Ruiz, Loreto Martorell, David Otaegui, Ander Matheu, Adolfo López de Munaín,
Describe the incidence of cancer in a large cohort of patients with myotonic dystrophy type 1 (DM1) and to unravel the underlying molecular mechanisms.Standardized incidence ratios (SIRs) were calculated in the Gipuzkoa DM1 cohort (1985-2013), dividing observed numbers by expected numbers for all cancers combined and stratified by sex. An estimation of the expected incidence was achieved by multiplying the age- and sex-specific incidence rates from the Basque population cancer registry by the person- ...
Tópico(s): RNA modifications and cancer
2016 - Lippincott Williams & Wilkins | Neurology
Beatríz Febrero, Antonio Ríos, A. López‐Navas, L. Martínez‐Alarcón, Javier Almela-Baeza, Á. Sánchez, José Luis Cobo Sánchez, Juan José Parrilla, Pablo Ramírez, Pascual Parrilla,
Abstract Background Teachers play a fundamental role in providing information to adolescents and could influence their attitudes. Objectives To analyze the attitude of teachers toward organ donation and the concept of brain death ( BD ); the psychosocial variables related to attitude; and the information provided to pupils. Methods A multicenter study was carried out using a random sample of secondary school teaching staff from 10 schools (n = 327) in the southeast of Spain. Attitude toward organ donation ...
Tópico(s): Organ Transplantation Techniques and Outcomes
2014 - Wiley | Clinical Transplantation
Matthew R. Donaldson, Judy L. Jensen, Martin Tristani‐Firouzi, Rabi Tawil, Saı̈d Bendahhou, William A. Suarez, Ana María Cobo, Juan José Poza, Elijah R. Behr, John Wagstaff, Pierre Szepetowski, Sandrine Pereira, Tahseen Mozaffar, Diana M. Escolar, Ying‐Hui Fu, Louis J. Ptáček,
Mutations in KCNJ2, the gene encoding the inward-rectifying K+ channel Kir2.1, cause the cardiac, skeletal muscle, and developmental phenotypes of Andersen-Tawil syndrome (ATS; also known as Andersen syndrome). Although pathogenic mechanisms have been proposed for select mutations, a common mechanism has not been identified.Seventeen probands presenting with symptoms characteristic of ATS were evaluated clinically and screened for mutations in KCNJ2. The results of mutation analysis were combined ...
Tópico(s): Amyotrophic Lateral Sclerosis Research
2003 - Lippincott Williams & Wilkins | Neurology
Amets Sáenz, France Leturcq, A.M. Cobo, Juan José Poza, Xavier Ferrer, David Otaegui, Pilar Camaño, Miguel Urtasun, Juan J. Vílchez, Eduardo Gutiérrez‐Rivas, José Ignacio Emparanza, Luciano Merlini, C. Paisán, María Goicoechea, Lorea Blázquez, B. Eymard, Hanns Lochmüller, Mathias C. Walter, C. Bönnemann, Dominique Figarella‐Branger, Josseline Kaplan, J. Andoni Urtizberea, J.F. Martí-Massó, Adolfo López de Munaín,
We present here the clinical, molecular and biochemical findings from 238 limb-girdle muscular dystrophy type 2A (LGMD2A) patients, representing ∼50% (238 out of 484) of the suspected calpainopathy cases referred for the molecular study of the calpain 3 (CAPN3) gene. The mean age at onset of LGMD2A patients was ∼14 years, and the first symptoms occurred between 6 and 18 years of age in 71% of patients. The mean age at which the patients became wheelchair bound was 32.2 years, with 84% requiring the use ...
Tópico(s): Nerve injury and regeneration
2005 - Oxford University Press | Brain
Isaiás García de la Fuenté, José Carlos Cobos, Juan Antonio González, Carlos Casanova, Marı́a José Cocero,
... enthalpies of dimethyl carbonate + hydrocarbons or + tetrachloromethaneIsaias Garcia, Jose C. Cobos, Juan A. Gonzalez, Carlos Casanova, and Maria J. CoceroCite ...
Tópico(s): Phase Equilibria and Thermodynamics
1988 - American Chemical Society | Journal of Chemical & Engineering Data
Forough Nazar Pour, Rebeca Cobos, Juan José R. Coque, João Serôdio, Artur Alves, Carina Félix, Vanessa Ferreira, Ana Cristina Esteves, Ana Sofia Duarte,
Neofusicoccum parvum is a fungal pathogen associated with a wide range of plant hosts. Despite being widely studied, the molecular mechanism of infection of N. parvum is still far from being understood. Analysis of N. parvum genome lead to the identification of six putative genes encoding necrosis and ethylene-inducing proteins (NLPs). The sequence of NLPs genes (NprvNep 1-6) were analyzed and four of the six NLP genes were successfully cloned, expressed in E. coli and purified by affinity chromatography. ...
Tópico(s): Yeasts and Rust Fungi Studies
2020 - Multidisciplinary Digital Publishing Institute | Toxins
Paula Alonso‐González, R. Cabo, I. San José, Ángel Gago, Iván Suazo Galdames, Olivia García‐Suárez, Juan Cobo, José A. Vega,
Ion channels are at the basis of the sensory processes including mechanosensing. Some members of the transient receptor potential (TRP) ion channel superfamily have been proposed as mechanosensors, but their putative role in mechanotransduction is controversial. Among them there are TRP canonical 6 (TRPC6) and TRP vanilloid 4 (TRPV4) ion channels, which are known to cooperate in mechanical hyperalgesia. Here, we investigated the occurrence, distribution, and possible colocalization of TRPC6 and TRPV4 ...
Tópico(s): Thermoregulation and physiological responses
2016 - Wiley | The Anatomical Record
Beatríz Febrero, Antonio Ríos, A. López‐Navas, L. Martínez‐Alarcón, Javier Almela-Baeza, José Luis Cobo Sánchez, Juan José Parrilla, Pascual Parrilla, Pablo Ramírez,
It is important for teenagers to have a favorable attitude toward organ donation and transplantation (ODT) in order to increase the number of future donors. To determine the attitude of teenagers in the South East of Spain toward ODT and to analyze the psychosocial variables related to this attitude.The study population consisted of young teenagers in the South East of Spain. A stratified sample was selected by geographical location, sex and age (n = 4, 117). Attitude was assessed using a validated ...
Tópico(s): Palliative Care and End-of-Life Issues
2019 - Oxford University Press | European Journal of Public Health
Maria Levanti, I. Esteban, E. Ciriaco, Pablo Pérez‐Piñera, R. Cabo, Olivia García‐Suárez, Berta Pardo, I. Silos-Santiago, Juan Cobo, José A. Vega,
The embryonic development of the enteric nervous system (ENS) from neural crest precursor cells requires neurotrophic signaling. Neurotrophins (NTs) are a family of growth factors that bind Trk receptors to signal diverse functions, including development and maintenance of different cell populations in the peripheral nervous system. In this study we investigated the expression and cell localization of TrkB, the high affinity receptor for brain-derived neurotrophic factor and NT-4, in the murine ENS ...
Tópico(s): Nerve injury and regeneration
2009 - Elsevier BV | Neuroscience Letters
M.G. Calavia, Jorge Feito, L. López-Iglesias, Félix de Carlos Villafranca, Olivia García‐Suárez, Pablo Pérez‐Piñera, Juan Cobo, José A. Vega,
Cutaneous Meissner corpuscles depend for development and survival exclusively on the NT system TrkB/BDNF/NT-4 unlike other types of sensory corpuscles and nerve endings, which have very complex neuronal and growth factor dependence. However, the pattern of expression of TrkB in human Meissner corpuscles is not known. The experiments in these studies were designed to pursue further findings that suggest that BDNF and NT-4 have critical roles in the development and maintenance of Meissner corpuscles ...
Tópico(s): Skin and Cellular Biology Research
2009 - Elsevier BV | Neuroscience Letters
Olivia García‐Suárez, Pablo Pérez‐Piñera, Rosaria Laurà, Antonino Germanà, I. Esteban, R. Cabo, I. Silos-Santiago, Juan Cobo, José A. Vega,
Normal development of the lung requires coordinated activation of cascades of signaling pathways initiated by growth factors signaling through their receptors. TrkB and its ligands, brain-derived neurotrophic factor (BDNF) and neurotrophin-4, belong to the neurotrophin family of growth factors, which are expressed in a large variety of non-neuronal tissues including the lung. Aberrant neurotrophin signaling underlies the pathogenesis of several lung-related pathologies, including asthma and lung ...
Tópico(s): Wnt/β-catenin signaling in development and cancer
2009 - Elsevier BV | Respiratory Physiology & Neurobiology
Olivia García‐Suárez, J.A Montano, I. Esteban, T. González‐Martínez, Covadonga Álvarez‐Abad, Elena López-Arranz, Juan Cobo, José A. Vega,
Abstract Myelinated nerve fibres forming sensory corpuscles become amyelinic before entering the corpuscle. Interestingly, in Meissner corpuscles from monkey myelin basic protein (MBP), a specific component of myelin sheath co‐localized with neuronal markers. To investigate whether or not this also occurs in human digital Meissner corpuscles, we used single and double immunohistochemistry to detect MBP associated with axonic (protein gene product (PGP) 9.5) or Schwann and Schwann‐related cell (S100 ...
Tópico(s): Skin and Cellular Biology Research
2009 - Wiley | Journal of Anatomy
J.A Montano, M.G. Calavia, Olivia García‐Suárez, J. A. Suárez-Quintanilla, Adriana Galvez, Pablo Pérez‐Piñera, Juan Cobo, José A. Vega,
Pacinian corpuscles are innervated by large myelinated Aα-β axons from the large- and intermediate-sized sensory neurons of dorsal root ganglia. These neurons express different members of the degenerin/epithelial Na+ channel (DEG/ENa+C) superfamily of proteins with putative mechanosensory properties, whose expression is regulated by the TrkB–BDNF system. Thus, we hypothesized that BDNF and/or NT-4 signalling through activation of TrkB may regulate the expression of molecules supposed to be necessary ...
Tópico(s): Ion Channels and Receptors
2009 - Elsevier BV | Neuroscience Letters
J.A Montano, Pablo Pérez‐Piñera, Olivia García‐Suárez, Juan Cobo, José A. Vega,
Abstract Null mutations of genes from the NGF family of NTs and their receptors (NTRs) lead to loss/reduction of specific neurons in sensory ganglia; conversely, cutaneous overexpression of NTs results in skin hyperinnervation and increase or no changes in the number of sensory neurons innervating the skin. These neuronal changes are paralleled with loss of specific types of sensory nerve formations in the skin. Therefore, mice carrying mutations in NT or NTR genes represent an ideal model to identify ...
Tópico(s): Cellular transport and secretion
2009 - Wiley | Microscopy Research and Technique
Pablo Pérez‐Piñera, Olivia García‐Suárez, Antonino Germanà, Belén Díaz‐Esnal, Félix de Carlos Villafranca, I. Silos-Santiago, Miguel del Valle Soto, Juan Cobo, José A. Vega,
The sensory deficit in TrkB deficient mice was evaluated by counting the neuronal loss in lumbar dorsal root ganglia (DRG), the absence of sensory receptors (cutaneous—associated to the hairy and glabrous skin - muscular and articular), and the percentage and size of the neurocalcin-positive DRG neurons (a calcium-binding protein which labels proprioceptive and mechanoceptive neurons). Mice lacking TrkB lost 32% of neurons, corresponding to the intermediate-sized and neurocalcin-positive ones. This neuronal ...
Tópico(s): Pain Mechanisms and Treatments
2008 - Elsevier BV | Neuroscience Letters
A. Solé-Magdalena, Enrique G. Revuelta, Ivan Menénez‐Díaz, M.G. Calavia, Teresa Cobo, Olivia García‐Suárez, Pablo Pérez‐Piñera, Félix de Carlos Villafranca, Juan Cobo, José A. Vega,
Abstract Diverse proteins of the denegerin/epithelial sodium channel (DEG/ENa + C) superfamily, in particular those belonging to the acid‐sensing ion channel (ASIC) family, as well as some members of the transient receptor protein (TRP) channel, function as mechanosensors or may be required for mechanosensation in a diverse range of species and cell types. Therefore, we investigated the putative mechanosensitive function of human odontoblasts using immunohistochemistry to detect ENa + C subunits (α, β, ...
Tópico(s): Ion channel regulation and function
2010 - Wiley | Microscopy Research and Technique
M.G. Calavia, J.A Montano, Olivia García‐Suárez, Jorge Feito, Marta Alonso Guervós, Antonino Germanà, Miguel del Valle Soto, Pablo Pérez‐Piñera, Juan Cobo, José A. Vega,
Tópico(s): Neurobiology and Insect Physiology Research
2010 - Springer Science+Business Media | Cellular and Molecular Neurobiology
Antonino Germanà, Simona Paruta, Germana P. Germanà, F. Javier Ochoa-Erena, Giuseppe Montalbano, Juan Cobo, José A. Vega,
Calcium-binding proteins play a critical role in vertebrate sensory cells, and some of them have been detected in mechanosensory and chemosensory cells of bony and cartilaginous fishes. In this study immunohistochemistry and Western blot were used to investigate the occurrence and the distribution of S100 protein and calretinin in mechanosensory (neuromasts of the lateral line system; maculae and cristae ampullaris of the inner ear) as well as chemosensory (superficial and oral taste buds; olfactory ...
Tópico(s): Immune Response and Inflammation
2007 - Elsevier BV | Brain Research
Yolanda García‐Mesa, Jorge García‐Piqueras, Beatriz García, Jorge Feito, R. Cabo, Juan Cobo, José A. Vega, Olivia García‐Suárez,
The transformation of mechanical energy into electrical signals is the first step in mechanotransduction in the peripheral sensory nervous system and relies on the presence of mechanically gated ion channels within specialized sensory organs called mechanoreceptors. Piezo2 is a vertebrate stretch-gated ion channel necessary for mechanosensitive channels in mammalian cells. Functionally, it is related to light touch, which has been detected in murine cutaneous Merkel cell-neurite complexes, Meissner- ...
Tópico(s): Ion Channels and Receptors
2017 - Wiley | Journal of Anatomy
Juan Cobo, Francesco Abbate, Juan Carlos de Vicente, Juan Cobo, José A. Vega,
The human craniofacial muscles innervated by the facial nerve typically lack muscle spindles. However these muscles have proprioception that participates in the coordination of facial movements. A functional substitution of facial proprioceptors by cutaneous mechanoreceptors has been proposed but at present this alternative has not been demonstrated. Here we have investigated whether other kinds of sensory structures are present in two human facial muscles (zygomatic major and buccal). Human checks ...
Tópico(s): Botulinum Toxin and Related Neurological Disorders
2017 - Elsevier BV | Neuroscience Letters
A. Solé-Magdalena, Mónica Martínez‐Alonso, César Coronado, Luís Junquera, Juan Cobo, José A. Vega,
Tópico(s): Biochemical Analysis and Sensing Techniques
2017 - Elsevier BV | Annals of Anatomy - Anatomischer Anzeiger
Jorge Feito, Olivia García‐Suárez, Jorge García‐Piqueras, Yolanda García‐Mesa, A. Pérez-Sánchez, Iván Suazo Galdames, R. Cabo, J. A. Suárez-Quintanilla, Juan Cobo, José A. Vega,
Meissner's and Pacinian corpuscles are cutaneous mechanoreceptors responsible for different modalities of touch. The development of these sensory formations in humans is poorly known, especially regarding the acquisition of the typical immunohistochemical profile related to their full functional maturity. Here we used a panel of antibodies (to specifically label the main corpuscular components: axon, Schwann-related cells and endoneurial-perineurial-related cells) to investigate the development of ...
Tópico(s): Hand Gesture Recognition Systems
2018 - Elsevier BV | Annals of Anatomy - Anatomischer Anzeiger
Ramón Cobo, Jorge García‐Piqueras, Juan Cobo, José A. Vega,
Sensory corpuscles of human skin are terminals of primary mechanoreceptive neurons associated with non-neuronal cells that function as low-threshold mechanoreceptors. Structurally, they consist of an extreme tip of a mechanosensory axon and nonmyelinating peripheral glial cells variably arranged according to the morphotype of the sensory corpuscle, all covered for connective cells of endoneurial and/or perineurial origin. Although the pathologies of sensitive corpuscles are scarce and almost never ...
Tópico(s): Botulinum Toxin and Related Neurological Disorders
2021 - Multidisciplinary Digital Publishing Institute | Journal of Clinical Medicine
Miguel del Valle Soto, Teresa Cobo, Juan Cobo, José A. Vega,
Abstract The mammalian skin has developed sensory structures (mechanoreceptors) that are responsible for different modalities of mechanosensitivity like touch, vibration, and pressure sensation. These specialized sensory organs are anatomically and functionally connected to a special subset of sensory neurons called mechanosensory neurons, which electrophysiologically correspond with Aβ fibers. Although mechanosensory neurons and cutaneous mechanoreceptors are rather well known, the biology of the ...
Tópico(s): Neurobiology and Insect Physiology Research
2012 - Wiley | Microscopy Research and Technique
Tatiana González-Martı x nez, Germana P. Germanà, Diego F. Monjil, Inmaculada Silos‐Santiago, Félix de Carlos Villafranca, G. Germanà, Juan Cobo, José A. Vega,
The TrkB-expressing sensory neurons seem to be involved in touch and other discriminative sensibilities. Thus, several slowly and rapidly adapting cutaneous mechanoreceptors, as well as muscle spindles, are reduced or absent in the territory of the trigeminal nerve in functionally TrkB-deficient mice. Whether this also occurs in the cutaneous or muscular territories of dorsal root ganglia has not been analyzed. Here we used immunohistochemistry and transmission-electron microscopy to analyze the ...
Tópico(s): Botulinum Toxin and Related Neurological Disorders
2004 - Elsevier BV | Brain Research
T. González‐Martínez, Isabel Fariñas, Miguel del Valle Soto, Jorge Feito, G. Germanà, Juan Cobo, José A. Vega,
Meissner corpuscles are rapidly adapting cutaneous mechanoreceptors depending for development on TrkB expressing sensory neurons, but it remains to be established which of the known TrkB ligands, BDNF or NT-4, is responsible of this dependence. In this study we analyze Meissner corpuscles in the digital pads of mice with target mutations in the genes encoding for either BDNF or NT-4, using immunohistochemistry and transmission-electron microscopy, and they were identified based on their morphology ...
Tópico(s): Dermatology and Skin Diseases
2004 - Elsevier BV | Neuroscience Letters
Tania Fontanil, Yamina Mohamedi, Ángela Moncada-Pazos, Teresa Cobo, José A. Vega, Juan Cobo, Olivia García‐Suárez, Juan Cobo, Álvaro J. Obaya, Santiago Cal,
The composition of the extracellular matrix (ECM) in the central nervous system (CNS) has several features that make it unique. For instance, it is remarkable for the presence of proteoglycans such as versican, brevican, and neurocan, some of which have been identified as substrates of different members of the ADAMTS family of secreted metalloproteases. Previous studies have associated ADAMTSs with the repair of the CNS, including recovery following degradation of glial scar tissue and the stimulation ...
Tópico(s): Signaling Pathways in Disease
2019 - Karger Publishers | Cellular Physiology and Biochemistry
Isaiás García de la Fuenté, Juan Antonio González, José Carlos Cobos, Carlos Casanova,
... benzene, toluene, or tetrachloromethaneIsaias Garcia de la Fuente, Juan A. Gonzalez, Jose C. Cobos, and Carlos CasanovaCite this: J. Chem. Eng. Data ...
Tópico(s): Chemical Thermodynamics and Molecular Structure
1992 - American Chemical Society | Journal of Chemical & Engineering Data
José-María Guerrero-Rodríguez, Clemente Cobos Sánchez, Juan José González de la Rosa, Diego Sales-Lérida,
The energy supply of office buildings and smart homes is a key issue in the global energy system. The growing use of microelectronics-based technology achieves new devices for a more comfortable life and wider use of electronic office equipment. On the one hand, these applications incorporate more and more sensitive electronic devices which are potentially affected by any external electrical transient. On the other hand, the existing electrical loads, which generally use electronic power systems ( ...
Tópico(s): Smart Grid Energy Management
2019 - Multidisciplinary Digital Publishing Institute | Energies
Ana María Cobo, Amets Sáenz, Juan José Poza, Miguel Urtasun, Begoña Indakoetxea, Jon Andoni Urtizberea, Adolfo López de Munaín, Francesc Calafell,
Limb-girdle muscular dystrophy type 2A (LGMD2A) is caused by any of over 150 mutations in the calpain-3 (CAPN3) gene. Of those, 2362AG → TCATCT is particularly prevalent in Basque patients, and this mutation was hypothesized to have arisen in the Basque Country. To explore the natural history of this mutation, we genotyped 65 Basque and non-Basque patients with LGMD2A who carry the 2362AG → TCATCT mutation for four microsatellites within or flanking the gene. A particular haplotype was found in three- ...
Tópico(s): Genetic Neurodegenerative Diseases
2004 - Wayne State University Press | Human Biology