Pathologic and Phenotypic Alterations in a Mouse Expressing a Connexin47 Missense Mutation That Causes Pelizaeus-Merzbacher–Like Disease in Humans

Gap junction channels are intercellular conduits that allow diffusional exchange of ions, second messengers, and metabolites. Human oligodendrocytes express the gap junction protein connexin47 (Cx47), which is encoded by the GJC2 gene. The autosomal recessive mutation hCx47M283T causes Pelizaeus-Merzbacher–like disease 1 (PMLD1), a progressive leukodystrophy characterized by hypomyelination, retarded motor development, nystagmus, and spasticity. We introduced the human missense mutation into the orthologous position of the mouse Gjc2 gene and inserted the mCx47M282T coding sequence into the mouse genome via homologous recombination in embryonic stem cells. Three-week-old homozygous Cx47M282T mice displayed impaired rotarod performance but unchanged open-field behavior. 10-15-day-old homozygous Cx47M282T and Cx47 null mice revealed a more than 80% reduction in the number of cells participating in glial networks after biocytin injections into oligodendrocytes in sections of corpus callosum. Homozygous expression of mCx47M282T resulted in reduced MBP expression and astrogliosis in the cerebellum of ten-day-old mice which could also be detected in Cx47 null mice of the same age. Three-month-old homozygous Cx47M282T mice exhibited neither altered open-field behavior nor impaired rotarod performance anymore. Adult mCx47M282T expressing mice did not show substantial myelin alterations, but homozygous Cx47M282T mice, additionally deprived of connexin32, which is also expressed in oligodendrocytes, died within six weeks after birth and displayed severe myelin defects accompanied by astrogliosis and activated microglia. These results strongly suggest that PMLD1 is caused by the loss of Cx47 channel function that results in impaired panglial coupling in white matter tissue

Datenreduzierter Digitaler HDTV-Recorder Abschlussbericht

The original goal was the development of a HDTV recorder within the EU 95 project. After the HD-MAC activities were cancelled this research project was decided into two subprojects: 1. datacompressed recording of digital video signals on disk. A lossless compression method was developed and implemented in a disk-recorder. 2. System solutions for the hierarchical steadiness of electronic filmscanning. Basic research activities were: improvement of the basic telecines stability; investigation of the principal deviations; extraction of reference points in dependence on the sprockethole and examination of the picture contents. An electronic correction method for the steadiness was developed. (orig.)SIGLEAvailable from TIB Hannover: F96B1004+a / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekBundesministerium fuer Bildung, Wissenschaft, Forschung und Technologie, Bonn (Germany)DEGerman

Variable Impedance Actuators: a Review

Variable Impedance Actuators (VIA) have received increasing attention in recent years as many novel applications involving interactions with an un- known and dynamic environment including humans require actuators with dynamics that are not well-achieved by classical stiff actuators. This paper presents an overview of the different VIAs developed and proposes a classification based on the principles through which the variable stiffness and damping are achieved. The main classes are active impedance by control, inherent compliance and damping actuators, inertial actuators, and combinations of them, which are then further divided into subclasses. This classification allows for designers of new devices to orientate and take inspiration and users of VIA’s to be guided in the design and implementation process for their targeted applicatio

Digitale Signalverarbeitung II Forschungsbericht

SIGLETIB: AC 9685 / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekDEGerman