Encuesta a estudiantes en actividad en la Facultad de Ciencias Económicas - UNLP - noviembre 2004 - febrero 2005

En el transcurso del año 2004, la Prosecretaría de Evaluación Permanente puso a consideración de las autoridades de la Facultad la conveniencia de profundizar la realización de encuestas teniendo en cuenta en principio lo dispuesto por la Ley Nº 24.521 del 7 de julio de 1995, que establece entre otras cosas: - que las instituciones universitarias deberán asegurar el funcionamiento de instancias internas de evaluación institucional que tendrán por objeto analizar los logros y dificultades en el cumplimiento de sus funciones, así como sugerir medidas para su mejoramiento. - que las encuestas a alumnos y graduados son instrumentos de evaluación importantes para la realimentación del proceso de enseñanza y aprendizaje. Las autoridades, entendiendo las razones expresadas por esta Prosecretaría, resolvieron mediante Resolución Nº 677/04 autorizar la realización periódica de encuestas para los alumnos de las carreras de grado de esta Facultad. En cumplimiento de lo dispuesto y como resultado del trabajo realizado en conjunto con investigadores del Departamento de Economía, el 1 de noviembre del 2004, se inició - con carácter obligatorio- una encuesta a los estudiantes en actividad con el objetivo de recolectar datos relativos a la composición, características sociales, demográficas y académicas del conjunto de los estudiantes así como conocer la opinión de los alumnos con respecto a servicios que brinda la facultad. La encuesta permitía el acceso tanto alumnos que cursan en la sede de La Plata como así también a aquellos alumnos de los centros regionales. La misma estaba conformada por cinco formularios, los que trataban sobre: Datos del estudiante y su familia (Formulario 1), Datos laborales y otras actividades (Formulario 2), Educación Secundaria (Formulario 3), Educación universitaria (Formulario 4) y Servicios de biblioteca e informáticos brindados por la Facultad (Formulario 5). Una copia de los formularios empleados se incluye en el Apéndice. Los datos resultados de la encuesta, que se presentan a continuación, incluyen un conjunto de informaciones elaboradas a partir del procesamiento de las respuestas válidas efectuadas por 4104 estudiantes de esta Facultad. Este número de alumnos representa el 62 % del total de votos sufragados en las elecciones de Claustro Estudiantil correspondientes al año 2004 y que se efectuaron los primeros días del mes de Noviembre de ese año. La presentación se ha organizado en cinco partes ordenadas por la temática abordada en cada uno de los formularios. Para una mejor comprensión de los datos consignados cabe tener presente que la encuesta permitía responder sobre cada uno de los formularios por separado, razón por la cual algunos de los estudiantes eligieron no contestar alguno de ellos y por lo tanto el total de respuestas por formulario difiere.Facultad de Ciencias Económicas (FCE

Animal models of episodic ataxia type 1 (EA1)

Episodic ataxia type 1 (EA1) is an autosomal dominant neurological disorder characterized by myokymia and attacks of ataxic gait often precipitated by stress. Several genetic mutations have been identified in the Shaker-like K+ channel Kv1.1 (KCNA1) of individuals with EA1. K+ channels are membrane proteins that allow the selected and concerted movement of K+ across a cell membrane that is otherwise relatively impermeable. Voltage-gated K+ channels shorten the duration of action potentials and control the excitability of central and peripheral neurons. EA1 is classified among ion channel diseases known as channelopathies (CPs). To date, a large group of CPs has been identified and new ones are continuously discovered. They result in a very diverse class of diseases ranging from ataxia, epilepsy, migraine, and psychiatric disorders to dysfunction of the skeletal muscle, kidney, and endocrinology system. Certainly, research using animal models of EA1 is providing important knowledge concerning the signaling pathways and circuits involved in this disease and in finding novel pharmacological interventions to ameliorate the symptoms. More broadly, investigations of CPs at the molecular and whole-animal levels will help further our understanding of the functional properties of ion channels and, eventually, the physiological workings of the human body.peer-reviewe

MKS3/TMEM67 mutations are a major cause of COACH syndrome, a joubert syndrome related disorder with liver involvement

The acronym COACH defines an autosomal recessive condition of Cerebellar vermis hypo/ aplasia, Oligophrenia, congenital Ataxia, Coloboma and Hepatic fibrosis. Patients present the “molar tooth sign”, a midbrain-hindbrain malformation pathognomonic for Joubert Syndrome (JS) and Related Disorders (JSRDs). The main feature of COACH is congenital hepatic fibrosis (CHF), resulting from malformation of the embryonic ductal plate. CHF is invariably found also in Meckel syndrome (MS), a lethal ciliopathy already found to be allelic with JSRDs at the CEP290 and RPGRIP1L genes. Recently, mutations in the MKS3 gene (approved symbol TMEM67), causative of about 7% MS cases, have been detected in few Meckel-like and pure JS patients. Analysis of MKS3 in 14 COACH families identified mutations in 8 (57%). Features such as colobomas and nephronophthisis were found only in a subset of mutated cases. These data confirm COACH as a distinct JSRD subgroup with core features of JS plus CHF, which major gene is MKS3, and further strengthen gene-phenotype correlates in JSRDs

Characterization of greater middle eastern genetic variation for enhanced disease gene discovery

The Greater Middle East (GME) has been a central hub of human migration and population admixture. The tradition of consanguinity, variably practiced in the Persian Gulf region, North Africa, and Central Asia1-3, has resulted in an elevated burden of recessive disease4. Here we generated a whole-exome GME variome from 1,111 unrelated subjects. We detected substantial diversity and admixture in continental and subregional populations, corresponding to several ancient founder populations with little evidence of bottlenecks. Measured consanguinity rates were an order of magnitude above those in other sampled populations, and the GME population exhibited an increased burden of runs of homozygosity (ROHs) but showed no evidence for reduced burden of deleterious variation due to classically theorized ‘genetic purging’. Applying this database to unsolved recessive conditions in the GME population reduced the number of potential disease-causing variants by four- to sevenfold. These results show variegated genetic architecture in GME populations and support future human genetic discoveries in Mendelian and population genetics

The node of Ranvier in CNS pathology.

Healthy nodes of Ranvier are crucial for action potential propagation along myelinated axons, both in the central and in the peripheral nervous system. Surprisingly, the node of Ranvier has often been neglected when describing CNS disorders, with most pathologies classified simply as being due to neuronal defects in the grey matter or due to oligodendrocyte damage in the white matter. However, recent studies have highlighted changes that occur in pathological conditions at the node of Ranvier, and at the associated paranodal and juxtaparanodal regions where neurons and myelinating glial cells interact. Lengthening of the node of Ranvier, failure of the electrically resistive seal between the myelin and the axon at the paranode, and retraction of myelin to expose voltage-gated K(+) channels in the juxtaparanode, may contribute to altering the function of myelinated axons in a wide range of diseases, including stroke, spinal cord injury and multiple sclerosis. Here, we review the principles by which the node of Ranvier operates and its molecular structure, and thus explain how defects at the node and paranode contribute to neurological disorders

Influence of control signals of the power converter operation

In the paper are presented the features of the control signals that affect on the work of the power bridge converter. The main purpose of the paper is to be done analysis on the features of the electronic switches and the circuits in a power converter and also to defines the requirements for their proper operation. These requirements must be satisfied by the electronic circuits for management of the power converter. In the paper is analyzed the operation of the power full bridge converter applied in mode of induction heating of metals. The researches in the paper are implementated in practical produced induction furnace. In the paper the simulation program PowerSim is used