Human deafness dystonia syndrome is a mitochondrial disease

The human deafness dystonia syndrome results from the mutation of a protein (DDP) of unknown function. We show now that DDP is a mitochondrial protein and similar to five small proteins (Tim8p, Tim9p, Tim10p, Tim12p, and Tim13p) of the yeast mitochondrial intermembrane space. Tim9p, Tim10p, and Tim12p mediate the import of metabolite transporters from the cytoplasm into the mitochondrial inner membrane and interact structurally and functionally with Tim8p and Tim13p. DDP is most similar to Tim8p. Tim8p exists as a soluble 70-kDa complex with Tim13p and Tim9p, and deletion of Tim8p is synthetically lethal with a conditional mutation in Tim10p. The deafness dystonia syndrome thus is a novel type of mitochondrial disease that probably is caused by a defective mitochondrial protein-import system.

The ATPase and protease domains of yeast mitochondrial Lon: Roles in proteolysis and respiration-dependent growth

The ATP-dependent Lon protease of Saccharomyces cerevisiae mitochondria is required for selective proteolysis in the matrix, maintenance of mitochondrial DNA, and respiration-dependent growth. Lon may also possess a chaperone-like function that facilitates protein degradation and protein-complex assembly. To understand the influence of Lon’s ATPase and protease activities on these functions, we examined several Lon mutants for their ability to complement defects of Lon-deleted yeast cells. We also developed a rapid procedure for purifying yeast Lon to homogeneity to study the enzyme’s activities and oligomeric state. A point mutation in either the ATPase or the protease site strongly inhibited the corresponding activity of the pure protein but did not alter the protein’s oligomerization; when expressed at normal low levels neither of these mutant enzymes supported respiration-dependent growth of Lon-deleted cells. When the ATPase- or the protease-containing regions of Lon were expressed as separate truncated proteins, neither could support respiration-dependent growth of Lon-deleted cells; however, coexpression of these two separated regions sustained wild-type growth. These results suggest that yeast Lon contains two catalytic domains that can interact with one another even as separate proteins, and that both are essential for the different functions of Lon.

Apontamentos críticos à distribuição dinâmica do ônus da prova

O ônus da prova tem sido tradicionalmente distribuído no processo civil brasileiro segundo disposições legais prévias, contidas em geral no artigo 333 do Código de Processo Civil e que em geral seguem os brocados jurídicos onus probandi incumbit ei qui allegat, probatio incumbit asserenti e semper necessitas probandi incumbit illi quit agit. Nos últimos anos, no entanto, tem crescido na doutrina e na jurisprudência a tendência de atribuir o onus probandi à parte que supostamente tem mais facilidade em produzir a prova nos autos, independentemente da distribuição predeterminada pela lei. A inspiração para esta mudança vem da teoria argentina das cargas probatórias dinâmicas, introduzida pelo juiz Jorge Peyrano e que teria suas raízes, supostamente, no trabalho de Jeremy Bentham. O projeto de um novo Código de Processo Civil, que está sendo discutido no Congresso Nacional, muito provavelmente incluirá disposição autorizado expressamente que o juiz desloque o ônus da prova de uma parte para a outra quando entender que esta última tem melhores condições de produzí-la. Os riscos invocados contra esta teoria são o aumento da insegurança jurídica, da possibilidade de arbitrariedade do julgador e da dificuldade de estabelecer previsões sobre sucesso processual, impedindo que as partes possam tomar as melhores decisões sobre como se portar antes e durante um eventual processo. Também há crítica contra o enfraquecimento da imparcialidade do juiz, o que, segundo os defensores da teoria, não ocorreria. Uma análise dos argumentos contra e a favor da teoria do ônus dinâmico da prova, dos instrumentos já existentes no direito brasileiro para os problemas que esta teoria vida atacar, e das novas disposições legais a serem em breve introduzidas demonstra que existe uma linha tênue a ser traçada e seguida para que se atinjam os benefícios pretendidos, sem cair em novos problemas. É importante adotar e interpretar as novas normas processuais cuidadosa e atenciosamente, de modo a evitar prejuízo a garantias básicas dos jurisdicionados.

Mathematical notebook, and commonplace book, circa 1689-1769

Manuscript notebook, possibly kept by Harvard students, containing 17th century English transcriptions of arithmetic and geometry texts, one of which is dated 1689-1690; 18th century transcriptions from John Ward’s “The Young Mathematician’s Guide”; and notes on physics lectures delivered by John Winthrop, the Hollis Professor of Mathematics and Natural Philosophy at Harvard from 1738 to 1779. The notebook also contains 18th century reading notes on Henry VIII, Tudor succession, and English history from Daniel Neal’s “The History of the Puritans” and David Hume’s “History of England,” and notes on Ancient history, taken mainly from Charles Rollin’s “The Ancient History of the Egyptians, Carthaginians, Assyrians, Babylonians, Medes and Persians, Macedonians and Grecians.” Additionally included are an excerpt from Plutarch’s “Lives” and transcriptions of three articles from “The Gentleman’s Magazine, and Historical Chronicle,” published in 1769: “A Critique on the Works of Ovid”; a book review of “A New Voyage to the West-Indies”; and “Genuine Anecdotes of Celebrated Writers, &.” The flyleaf contains the inscription “Semper boni aliquid operis facito ut diabolus te semper inveniat occupatum,” a variation on a quote of Saint Jerome that translates approximately as “Always good to do some work so that the devil may always find you occupied.” In the seventeenth and eighteenth centuries, Harvard College undergraduates often copied academic texts and lecture notes into personal notebooks in place of printed textbooks. Winthrop used Ward’s textbook in his class, while the books of Hume, Neal, and Rollin were used in history courses taught at Harvard in the 18th century.

Karte des Bosporus und der Bithynischen Halbinsel

nach älteren Quellen (v. Moltke, Heinrich Kiepert, Brit. Admiralitätskarten, v. d. Goltz, v. Diest u.a.) und eigenen Aufnahmen im Jahre 1900 gezeichnet von Rudolf Fitzner ; Hof. Stdr. v. J. G. Tiedmann Nachf.

Imperium Turcicum per Europam, Asiam Et Africam se extendens et secundum Beglerbegatus sive Praefecturas accurate distinctum

Covers Libya, Egypt, Sudan, the Arabian Peninsula, Lebanon, Syria, Iraq, Iran, the Balkan Peninsula, Turkey, Greece, Armenia, Azerbaijan and portions of Ethiopia and Russia.

Scaling Social Impact in Europe: Quantitative Analysis of National and Transnational Scaling Strategies of 358 Social Enterprises

A wide variety of different socially motivated organisations perform exceptional feats in alleviating societal ills using creativity and entrepreneurial spirit in their quest to scale their social impact. Their focus is seldom limited to one specific region – instead they strive to benefit the largest possible number of people, thereby often transcending national borders. After all, societal issues usually don’t stop at a countries’ borders – why then should good ideas and impactful concepts aimed at solving these issues? For many socially motivated organisations, the goal of disseminating their social impact remains a solemn wish due to lacking experience and know-how of how to plan and conduct systematic scaling.

Temporal Control of Leaf Complexity by miRNA-Regulated Licensing of Protein Complexes

The tremendous diversity of leaf shapes has caught the attention of naturalists for centuries. In addition to interspecific and intraspecific differences, leaf morphologies may differ in single plants according to age, a phenomenon known as heteroblasty. In Arabidopsis thaliana, the progression from the juvenile to the adult phase is characterized by increased leaf serration. A similar trend is seen in species with more complex leaves, such as the A. thaliana relative Cardamine hirsuta, in which the number of leaflets per leaf increases with age. Although the genetic changes that led to the overall simpler leaf architecture in A. thaliana are increasingly well understood, less is known about the events underlying age-dependent changes within single plants, in either A. thaliana or C. hirsuta. Here, we describe a conserved miRNA transcription factor regulon responsible for an age-dependent increase in leaf complexity. In early leaves, miR319-targeted TCP transcription factors interfere with the function of miR164-dependent and miR164-independent CUC proteins, preventing the formation of serrations in A. thaliana and of leaflets in C. hirsuta. As plants age, accumulation of miR156-regulated SPLs acts as a timing cue that destabilizes TCP-CUC interactions. The destabilization licenses activation of CUC protein complexes and thereby the gradual increase of leaf complexity in the newly formed organs. These findings point to posttranslational interaction between unrelated miRNA-targeted transcription factors as a core feature of these regulatory circuits.

Geschichte und merkwürdigkeiten der Stiftsbibliothek in Zeiz. Als einladung zu einer redeübung in der Stiftsschule zu Zeiz den 16. may 1808.

Mode of access: Internet.

Thesaurus literaturae theologicae academicae; sive, Rcensus dissertationum, programmatum ... cum delectu uberrimo scriptionum academicarum, philologicarum ... quae ab antiquissimis usque ad recentissimia tempora editae in collectione Goethiana Lipsiensi sunt venales.

No more published.

Cuentos nerviosos.

Por qué la mató.- Catalepsia.- El primer esclavo.- Sub lumine semper.- La autopsia.- Una duda.- La muerte del "Maestro." - El centinela.- Cavilaciones.- El viejo maestro.- Maldita! - At home.- Madonna mía! - Confidencias.- El vengador.- Guitarras y fusiles.

Sämmtliche Werke.

Vol. 1. Erläuterungen und Ergänzungen zum Wesen des Christenthums.- Vol. 2. Philosophische Kritiken und Grundsätze.- Vol. 3. Gedanken über Tod und Unsterblichkeit.- Vol. 4. Geschichte der neuern Philosophie von Bacon von Verulam bis Benedict Spinoza.- Vol. 5. Darstellung, Entwicklung und Kritik der Leibnitz'schen Philosophie.- Vol. 6. Pierre Bayle, ein Beitrag zur Geschichte der Philosophie und Menschheit.- Vol. 7. Das Wesen des Christenthums.- Vol. 8. Vorlesungen über das wesen der Religion.- Vol. 9. Theogonie nachder Quellen des classisischen, hebräischen und christlichen Alterthums.- Vol. 10. Gottheit, Freiheit und Unsterblichkeit vom Standpunkte der Anthropologie.

Beschreibung der groszen Orgel der Marienkirche zu Wismar sowie der groszen Orgel des Domes und der St. Martinikirche zu Halberstadt : ein Beitrag zur Beleuchtung und Würdigung der eigenthümlichen Ansichten und Grundsätze des Herrn Musikdirectors Wilke zu Neu-Ruppin in Bezug auf die Orgelbaukunst von Ferdinand Baake.

Contains history and description of organs built by Friedrich Schulze, David Beck, Josef Wiedemann, and Johann Gottlob Töpfer.

Catalogus bibliothecae numerosae ab incluti nominis viro Hieronymo Guilielmo Ebnero, ab Eschenbach rel. olim conlectae, nunc Norimbergae ... ann. MDCCCXIII[-MDCCCXX] publicae auctionis lege diuendendae. Quem in hunc ordinem redegit, his literarii maximam partem generis notationibus instruxit,

Vol. 1 has added t.-p. (with portrait vignette): Bibliotheca Ebneriana ...