Situación socio familiar y perspectivas profesionales en estudiantes de Artes Plásticas de la ESFAP y la UNA de Puno 2017

El objetivo de la investigación fue identificar la relación entre la situación socio familiar con las perspectivas profesionales de alumnos de artes plásticas de la ESFAP Puno y la escuela de arte de la UNA Puno en el año 2017, el estudio fue descriptivo con diseño relacional. Se utilizó una muestra de estudiantes de la ESFAP Puno del primero al décimo semestre de 18 y de la UNA Puno 24 estudiantes del primero al décimo semestre, el instrumento utilizado fue dos encuestas, una de aspectos socio familiar y otra de perspectivas profesionales. Los resultados fueron: El análisis estadístico de correlación, señala que el coeficiente fue r=0.635, el signo positivo indica que al mejorar la situación socio familiar las perspectivas profesionales son buenas en los alumnos de artes plásticas de la ESFAP-Puno, la correlación es estadísticamente significativa con un 95% de confianza (α=0.05). En estudiantes de la UNA Puno la relación entre las variables factores socio familiares y perspectivas profesionales en alumnos de la UNA Puno. Son también positivas, el análisis estadístico de correlación, señala que el coeficiente fue r=0.443, el signo positivo indica que al incrementar los factores socio familiar las perspectivas profesionales mejoran en los alumnos de Artes Plásticas de la UNA-Puno, la correlación es estadísticamente significativa con un 95% de confianza (α=0.05). Al término del estudio se demostró la hipótesis planteada que indica que la situación socio familiar y las perspectivas profesionales tienen una relación positiva.Tesi

Interplay of RFX transcription factors 1, 2 and 3 in motile ciliogenesis

Cilia assembly is under strict transcriptional control during animal development. In vertebrates, a hierarchy of transcription factors (TFs) are involved in controlling the specification, differentiation and function of multiciliated epithelia. RFX TFs play key functions in the control of ciliogenesis in animals. Whereas only one RFX factor regulates ciliogenesis in C. elegans, several distinct RFX factors have been implicated in this process in vertebrates. However, a clear understanding of the specific and redundant functions of different RFX factors in ciliated cells remains lacking. Using RNA-seq and ChIP-seq approaches we identified genes regulated directly and indirectly by RFX1, RFX2 and RFX3 in mouse ependymal cells. We show that these three TFs have both redundant and specific functions in ependymal cells. Whereas RFX1, RFX2 and RFX3 occupy many shared genomic loci, only RFX2 and RFX3 play a prominent and redundant function in the control of motile ciliogenesis in mice. Our results provide a valuable list of candidate ciliary genes. They also reveal stunning differences between compensatory processes operating in vivo and ex vivo

Targeted NGS gene panel identifies mutations in RSPH1 causing primary ciliary dyskinesia and a common mechanism for ciliary central pair agenesis due to radial spoke defects.

Primary ciliary dyskinesia (PCD) is an inherited chronic respiratory obstructive disease with randomized body laterality and infertility, resulting from cilia and sperm dysmotility. PCD is characterized by clinical variability and extensive genetic heterogeneity, associated with different cilia ultrastructural defects and mutations identified in >20 genes. Next generation sequencing (NGS) technologies therefore present a promising approach for genetic diagnosis which is not yet in routine use. We developed a targeted panel-based NGS pipeline to identify mutations by sequencing of selected candidate genes in 70 genetically undefined PCD patients. This detected loss-of-function RSPH1 mutations in four individuals with isolated central pair (CP) agenesis and normal body laterality, from two unrelated families. Ultrastructural analysis in RSPH1-mutated cilia revealed transposition of peripheral outer microtubules into the 'empty' CP space, accompanied by a distinctive intermittent loss of the central pair microtubules. We find that mutations in RSPH1, RSPH4A and RSPH9, which all encode homologs of components of the 'head' structure of ciliary radial spoke complexes identified in Chlamydomonas, cause clinical phenotypes that appear to be indistinguishable except at the gene level. By high-resolution immunofluorescence we identified a loss of RSPH4A and RSPH9 along with RSPH1 from RSPH1-mutated cilia, suggesting RSPH1 mutations may result in loss of the entire spoke head structure. CP loss is seen in up to 28% of PCD cases, in whom laterality determination specified by CP-less embryonic node cilia remains undisturbed. We propose this defect could arise from instability or agenesis of the ciliary central microtubules due to loss of their normal radial spoke head tethering

Survey of the ciliary motility machinery of Drosophila sperm and ciliated mechanosensory neurons reveals unexpected cell-type specific variations: a model for motile ciliopathies

The motile cilium/flagellum is an ancient eukaryotic organelle. The molecular machinery of ciliary motility comprises a variety of cilium-specific dynein motor complexes along with other complexes that regulate their activity. Assembling the motors requires the function of dedicated “assembly factors” and transport processes. In humans, mutation of any one of at least 40 different genes encoding components of the motility apparatus causes Primary Ciliary Dyskinesia (PCD), a disease of defective ciliary motility. Recently, Drosophila has emerged as a model for motile cilia biology and motile ciliopathies. This is somewhat surprising as most Drosophila cells lack cilia, and motile cilia are confined to just two specialized cell types: the sperm flagellum with a 9+2 axoneme and the ciliated dendrite of auditory/proprioceptive (chordotonal, Ch) neurons with a 9+0 axoneme. To determine the utility of Drosophila as a model for motile cilia, we survey the Drosophila genome for ciliary motility gene homologs, and assess their expression and function. We find that the molecules of cilium motility are well conserved in Drosophila. Most are readily characterized by their restricted cell-type specific expression patterns and phenotypes. There are also striking differences between the two motile ciliated cell types. Notably, sperm and Ch neuron cilia express and require entirely different outer dynein arm variants—the first time this has been clearly established in any organism. These differences might reflect the specialized functions for motility in the two cilium types. Moreover, the Ch neuron cilia lack the critical two-headed inner arm dynein (I1/f) but surprisingly retain key regulatory proteins previously associated with it. This may have implications for other motile 9+0 cilia, including vertebrate embryonic nodal cilia required for left-right axis asymmetry. We discuss the possibility that cell-type specificity in ciliary motility machinery might occur in humans, and therefore underlie some of the phenotypic variation observed in PCD caused by different gene mutations. Our work lays the foundation for the increasing use of Drosophila as an excellent model for new motile ciliary gene discovery and validation, for understanding motile cilium function and assembly, as well as understanding the nature of genetic defects underlying human motile ciliopathies

Functional characterization of two new ciliary genes during the development of vertebrate

Les cils et les flagelles sont des organites cellulaires très conservés qui assurent des fonctions essentielles. Chez l'Homme, les défauts d'assemblage des cils et des flagelles conduisent à de multiples pathologies, les ciliopathies. Afin de comprendre comment se forment et fonctionnent les cils, j'ai analysé la fonction de deux nouveaux gènes identifiés comme cible des facteurs de transcription de ciliogenèse RFX. Tout d'abord je me suis focalisée sur le gène CCDC151, évolutivement conservé dans les espèces possédant des cils motiles. J'ai pu montrer que CCDC151 est impliquée dans le transport dépendant de l'IFT des bras de dynéine chez les animaux et qu'elle est nécessaire à la perception sensorielle chez la drosophile. Par ailleurs, j'ai également montré que cette protéine possède des fonctions cellulaires additionnelles puisqu'elle est requise pour l'orientation correcte des plans de division cellulaire et qu'elle est impliquée dans la régulation de la taille du cil primaire chez les mammifères. Je me suis ensuite intéressée au gène LRRC48 également conservée dans les espèces possédant des cils motiles. Cette protéine est nécessaire à la motilité des flagelles de spermatozoïdes et des cils des neurones sensoriels en 9+0 et dans la réponse auditive chez la drosophile. De plus LRRC48 est indispensable au développement des vertébrés puisque son absence chez le poisson zèbre conduit à l'hydrocéphalie, des kystes rénaux et des défauts de motilité des cils. Elle est également essentielle à la biogenèse de l'oreille dans cet organisme.En conclusion, il s'agit de deux nouveaux acteurs de la ciliogenèse potentiellement impliqués dans les pathologies ciliaires chez l'HommeCilia are highly conserved structures found from protozoa to mammals where they play essential physiological and developmental functions and cilia dysfunction leads to various syndromes in humans known as ciliopathies. To understand cilia formation and function, I performed functional analysis of two new target genes of the RFX ciliogenic transcription factors. First, I focused on CCDC151 that is evolutionary conserved in motile ciliated species. I showed that CCDC151 is involved in the control of IFT-dependent dynein arm assembly in animals and required for geotaxis behavior of adult flies. In zebrafish, depletion of Ccdc151 leads to left-right asymmetry defects and kidney cysts, two phenotypes resulting from impaired ciliary beating. However, I also showed that CCDC151 is also implicated in other cellular functions in vertebrates as it is involved in proper orientation of cell divisions and implicated in the regulation of primary cilium length in mammalian cells. In a second part, I studied LRRC48 that is also conserved in species with motile cilia. I showed that this protein is essential for motility of flagellar spermatozoids and for motility of the 9+0 sensory cilia as well as in the auditory response in drosophila. In zebrafish, morpholinos induced depletion of this protein leads to hydrocephaly, kidney cysts, inner ear abnormalities and cilia motility defects. Moreover this protein is also required for inner ear biogenesis in the model. In conclusion, these two genes are essential for ciliogenesis and they are new candidate genes potentially implicated in human ciliary disease

Influência da participação do mercado na remuneração da estrutura de capital: uma analise de empresas do varejo automobilístico na perspectiva do EVA®

Dissertação (mestrado) - Universidade do Estado de Santa Catarina, Centro Tecnológico. Programa de Pós-Graduação em Engenharia de ProduçãoO objetivo do trabalho é verificar a influência da participação do mercado na remuneração da estrutura de capital, na perspectiva do Economic Value Added (Valor Econômico Agregado) EVA®, em empresas do varejo automobilística da marca Alfa estabelecidas em Santa Catarina. A metodologia empregada na presente pesquisa é descritiva do tipo levantamento. Para tanto, da população de empresas, foi tomada uma amostra não probabilística por tipicidade ou intencional, abrangendo empresas do varejo automobilístico do Estado de Santa Catarina, totalizando 23 demonstrações contábeis relativas ao ano de 2000 a 2003. As informações contábeis foram organizadas por regiões operacionais, na mesma forma de divisão geográfica de Santa Catarina, seguida pela montadora marca da Alfa, sendo que, o Estado é dividido em 21 regiões operacionais. Em seguida, foram feitos os testes de correlação entre as variáveis estabelecidas para alcançar os objetivos propostos. A partir da pesquisa, observou-se na análise de correlação entre as variáveis, participação no mercado e remuneração da estrutura de capital na perspectiva EVA®, uma evidência de fraca correlação significativa para os períodos analisados. Os resultados da pesquisa apresentam subsídios para compreender o reflexo do custo do capital total nos resultados das empresas. Oferece informações para os gestores que a composição da estrutura de capital, com elevada participação de capital próprio, evidencia EVAs® negativos. Ressalta que em mercados com limites de escalas, como é o caso de Santa Catarina, o bom posicionamento da empresa como líder em seu mercado, não significa gerar fluxos positivos de EVA®

Caractérisation fonctionnelle de deux nouveaux gènes ciliaires pendant le développement des vertébrés

Cilia are highly conserved structures found from protozoa to mammals where they play essential physiological and developmental functions and cilia dysfunction leads to various syndromes in humans known as ciliopathies. To understand cilia formation and function, I performed functional analysis of two new target genes of the RFX ciliogenic transcription factors. First, I focused on CCDC151 that is evolutionary conserved in motile ciliated species. I showed that CCDC151 is involved in the control of IFT-dependent dynein arm assembly in animals and required for geotaxis behavior of adult flies. In zebrafish, depletion of Ccdc151 leads to left-right asymmetry defects and kidney cysts, two phenotypes resulting from impaired ciliary beating. However, I also showed that CCDC151 is also implicated in other cellular functions in vertebrates as it is involved in proper orientation of cell divisions and implicated in the regulation of primary cilium length in mammalian cells. In a second part, I studied LRRC48 that is also conserved in species with motile cilia. I showed that this protein is essential for motility of flagellar spermatozoids and for motility of the 9+0 sensory cilia as well as in the auditory response in drosophila. In zebrafish, morpholinos induced depletion of this protein leads to hydrocephaly, kidney cysts, inner ear abnormalities and cilia motility defects. Moreover this protein is also required for inner ear biogenesis in the model. In conclusion, these two genes are essential for ciliogenesis and they are new candidate genes potentially implicated in human ciliary diseasesLes cils et les flagelles sont des organites cellulaires très conservés qui assurent des fonctions essentielles. Chez l'Homme, les défauts d'assemblage des cils et des flagelles conduisent à de multiples pathologies, les ciliopathies. Afin de comprendre comment se forment et fonctionnent les cils, j'ai analysé la fonction de deux nouveaux gènes identifiés comme cible des facteurs de transcription de ciliogenèse RFX. Tout d'abord je me suis focalisée sur le gène CCDC151, évolutivement conservé dans les espèces possédant des cils motiles. J'ai pu montrer que CCDC151 est impliquée dans le transport dépendant de l'IFT des bras de dynéine chez les animaux et qu'elle est nécessaire à la perception sensorielle chez la drosophile. Par ailleurs, j'ai également montré que cette protéine possède des fonctions cellulaires additionnelles puisqu'elle est requise pour l'orientation correcte des plans de division cellulaire et qu'elle est impliquée dans la régulation de la taille du cil primaire chez les mammifères. Je me suis ensuite intéressée au gène LRRC48 également conservée dans les espèces possédant des cils motiles. Cette protéine est nécessaire à la motilité des flagelles de spermatozoïdes et des cils des neurones sensoriels en 9+0 et dans la réponse auditive chez la drosophile. De plus LRRC48 est indispensable au développement des vertébrés puisque son absence chez le poisson zèbre conduit à l'hydrocéphalie, des kystes rénaux et des défauts de motilité des cils. Elle est également essentielle à la biogenèse de l'oreille dans cet organisme.En conclusion, il s'agit de deux nouveaux acteurs de la ciliogenèse potentiellement impliqués dans les pathologies ciliaires chez l'Homm

Situación socio familiar y perspectivas profesionales en estudiantes de Artes Plásticas de la ESFAP y la UNA de Puno 2017

TesisEl objetivo de la investigación fue identificar la relación entre la situación socio familiar con las perspectivas profesionales de alumnos de artes plásticas de la ESFAP Puno y la escuela de arte de la UNA Puno en el año 2017, el estudio fue descriptivo con diseño relacional. Se utilizó una muestra de estudiantes de la ESFAP Puno del primero al décimo semestre de 18 y de la UNA Puno 24 estudiantes del primero al décimo semestre, el instrumento utilizado fue dos encuestas, una de aspectos socio familiar y otra de perspectivas profesionales. Los resultados fueron: El análisis estadístico de correlación, señala que el coeficiente fue r=0.635, el signo positivo indica que al mejorar la situación socio familiar las perspectivas profesionales son buenas en los alumnos de artes plásticas de la ESFAP-Puno, la correlación es estadísticamente significativa con un 95% de confianza (α=0.05). En estudiantes de la UNA Puno la relación entre las variables factores socio familiares y perspectivas profesionales en alumnos de la UNA Puno. Son también positivas, el análisis estadístico de correlación, señala que el coeficiente fue r=0.443, el signo positivo indica que al incrementar los factores socio familiar las perspectivas profesionales mejoran en los alumnos de Artes Plásticas de la UNA-Puno, la correlación es estadísticamente significativa con un 95% de confianza (α=0.05). Al término del estudio se demostró la hipótesis planteada que indica que la situación socio familiar y las perspectivas profesionales tienen una relación positiva