Ichthyosen: Pathophysiologische Modelle der epidermalen Differenzierung

Zusammenfassung: Die Ichthyosen sind eine durch Schuppung oder Keratosen charakterisierte heterogene Krankheitsgruppe monogenetisch vererbter Verhornungsstörungen. In der Vergangenheit wurden sie aufgrund klinischer Zeichen und des Vererbungsmusters nosologisch eingeteilt. Als Folge des massiven Wissenssprunges im Gebiet der molekularen Genetik wurden nun zahlreiche Gendefekte im Gebiet der Ichthyosen aufgeklärt. Dadurch trat die Komplexität der Ichthyosen zutage, wobei ähnliche oder gleiche Phänotypen durch Mutationen in verschiedenen Genen verursacht werden können, währenddessen umgekehrt einzelne Gene in verschiedenen, zum Teil stark divergierenden Phänotypen resultieren können. Daher wurde eine neue Klassifizierung notwendig, die klinische und neue molekulargenetische Erkenntnisse berücksichtigt und vereint. In dieser Publikation folgen wir größtenteils der Einteilung, die anlässlich der ersten Ichthyosis Consensus Conference in Sorèze, Frankreich, festgelegt wurd

TRAIP is a regulator of the spindle assembly checkpoint.

Accurate chromosome segregation during mitosis is temporally and spatially coordinated by fidelity-monitoring checkpoint systems. Deficiencies in these checkpoint systems can lead to chromosome segregation errors and aneuploidy, and promote tumorigenesis. Here, we report that the TRAF-interacting protein (TRAIP), a ubiquitously expressed nucleolar E3 ubiquitin ligase important for cellular proliferation, is localized close to mitotic chromosomes. Its knockdown in HeLa cells by RNA interference (RNAi) decreased the time of early mitosis progression from nuclear envelope breakdown (NEB) to anaphase onset and increased the percentages of chromosome alignment defects in metaphase and lagging chromosomes in anaphase compared with those of control cells. The decrease in progression time was corrected by the expression of wild-type but not a ubiquitin-ligase-deficient form of TRAIP. TRAIP-depleted cells bypassed taxol-induced mitotic arrest and displayed significantly reduced kinetochore levels of MAD2 (also known as MAD2L1) but not of other spindle checkpoint proteins in the presence of nocodazole. These results imply that TRAIP regulates the spindle assembly checkpoint, MAD2 abundance at kinetochores and the accurate cellular distribution of chromosomes. The TRAIP ubiquitin ligase activity is functionally required for the spindle assembly checkpoint control

Разработка способов, алгоритмов и программ обнаружения и исправления пакетных или независимых ошибок при передаче данных по каналам связи

Настоящая научно-квалификационная работа посвящена разработке способов, алгоритмов и программ обнаружения и исправления ошибок при передаче данных по каналам связи для обеспечения целостности и надёжности передаваемых команд и данных между устройствами вычислительно техники, а также при считывании данных из запоминающих устройств.The present scientific and qualification work is devoted to the development of methods, algorithms and programs for detecting and correcting errors in the transmission of data over communication channels to ensure the integrity and reliability of the transmitted commands and data between computing devices, as well as when reading data from memory devices

Late-Onset Multiple Self-Healing Squamous Epithelioma Ferguson-Smith Recurrence Induced by Radiotherapy.

We report the case of a woman in her 60s with confirmed multiple self-healing squamous epitheliomas (MSSE) Ferguson-Smith. After recurrences following surgery and radiotherapy, the patient was successfully treated with minimal surgical intervention combined with intralesional injections of triamcinolone acetate. The histological comparison between mature and regressed keratoacanthomas (KA) revealed an increased inflammatory infiltrate with numerous plasmacytoid dendritic cells in the regressed KA in comparison to the mature one, speaking in favor of an inflammation-mediated regression process. Corticosteroids injection in MSSE may have paradoxical effects with action on the proliferation phase rather than the inflammatory regression phase of the KA. Our case confirms previous reports showing that radiotherapy may exacerbate MSSE and should be avoided. Intralesional triamcinolone acetate injection is a safe and easy to use method also effective for multiple lesions. Our case underlines the difference between squamous cell carcinoma and KA, responding differently to therapies like imiquimod or radiotherapy

Secular Evolution of Galaxy Morphologies

Today we have numerous evidences that spirals evolve dynamically through various secular or episodic processes, such as bar formation and destruction, bulge growth and mergers, sometimes over much shorter periods than the standard galaxy age of 10-15 Gyr. This, coupled to the known properties of the Hubble sequence, leads to a unique sense of evolution: from Sm to Sa. Linking this to the known mass components provides new indications on the nature of dark matter in galaxies. The existence of large amounts of yet undetected dark gas appears as the most natural option. Bounds on the amount of dark stars can be given since their formation is mostly irreversible and requires obviously a same amount of gas.Comment: 8 pages, Latex2e, crckapb.sty macros, 1 Postscript figure, replaced with TeX source; To be published in the proceeedings of the "Dust-Morphology" conference, Johannesburg, 22-26 January, 1996, D. Block (ed.), (Kluwer Dordrecht

Experimental Evidence of Time Delay Induced Death in Coupled Limit Cycle Oscillators

Experimental observations of time delay induced amplitude death in a pair of coupled nonlinear electronic circuits that are individually capable of exhibiting limit cycle oscillations are described. In particular, the existence of multiply connected death islands in the parameter space of the coupling strength and the time delay parameter for coupled identical oscillators is established. The existence of such regions was predicted earlier on theoretical grounds in [Phys. Rev. Lett. 80, 5109 (1998); Physica 129D, 15 (1999)]. The experiments also reveal the occurrence of multiple frequency states, frequency suppression of oscillations with increased time delay and the onset of both in-phase and anti-phase collective oscillations.Comment: 4 aps formatted RevTeX pages; 6 figures; to appear in Phys. Rev. Let

Ab initio simulations of liquid systems: Concentration dependence of the electric conductivity of NaSn alloys

Liquid NaSn alloys in five different compositions (20, 40, 50, 57 and 80% sodium) are studied using density functional calculations combined with molecular dynamics(Car-Parrinello method). The frequency-dependent electric conductivities for the systems are calculated by means of the Kubo-Greenwood formula. The extrapolated DC conductivities are in good agreement with the experimental data and reproduce the strong variation with the concentration. The maximum of conductivity is obtained, in agreement with experiment, near the equimolar composition. The strong variation of conductivity, ranging from almost semiconducting up to metallic behaviour, can be understood by an analysis of the densities-of-states.Comment: LaTex 6 pages and 2 figures, to appear in J.Phys. Cond. Ma

Влияние ширины реза на радиальную проекцию силы резания

В статье обосновывается влияние ширины реза на проекцию составляющей силы резания на плоскость, перпендикулярную оси вращения геохода. Рассмотрена актуальность исследования. Для постановки цели и задач исследования определена проекция составляющей силы резания на плоскость, перпендикулярную оси вращения геохода. На основании проведенного исследования построена зависимость радиальной проекции силы резанию ножевого исполнительного органа геохода (RИО.СВ) от от x до Rг