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See detailLoss-of-function mutations in ATP6V0A2 impair vesicular trafficking, tropoelastin secretion and cell survival.
Hucthagowder, Vishwanathan; Morava, Eva; Kornak, Uwe et al

in Human Molecular Genetics (2009), 18(12), 2149-65

Autosomal recessive cutis laxa type 2 (ARCL2), a syndrome of growth and developmental delay and redundant, inelastic skin, is caused by mutations in the a2 subunit of the vesicular ATPase H+-pump ... [more ▼]

Autosomal recessive cutis laxa type 2 (ARCL2), a syndrome of growth and developmental delay and redundant, inelastic skin, is caused by mutations in the a2 subunit of the vesicular ATPase H+-pump (ATP6V0A2). The goal of this study was to define the disease mechanisms that lead to connective tissue lesions in ARCL2. In a new cohort of 17 patients, DNA sequencing of ATP6V0A2 detected either homozygous or compound heterozygous mutations. Considerable allelic and phenotypic heterogeneity was observed, with a missense mutation of a moderately conserved residue p.P87L leading to unusually mild disease. Abnormal N- and/or mucin type O-glycosylation was observed in all patients tested. Premature stop codon mutations led to decreased ATP6V0A2 mRNA levels by destabilizing the mutant mRNA via the nonsense-mediated decay pathway. Loss of ATP6V0A2 either by siRNA knockdown or in ARCL2 cells resulted in distended Golgi cisternae, accumulation of abnormal lysosomes and multivesicular bodies. Immunostaining of ARCL2 cells showed the accumulation of tropoelastin (TE) in the Golgi and in large, abnormal intracellular and extracellular aggregates. Pulse-chase studies confirmed impaired secretion and increased intracellular retention of TE, and insoluble elastin assays showed significantly reduced extracellular deposition of mature elastin. Fibrillin-1 microfibril assembly and secreted lysyl oxidase activity were normal in ARCL2 cells. TUNEL staining demonstrated increased rates of apoptosis in ARCL2 cell cultures. We conclude that loss-of-function mutations in ATP6V0A2 lead to TE aggregation in the Golgi, impaired clearance of TE aggregates and increased apoptosis of elastogenic cells. [less ▲]

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See detailLosses – Marks&Spencer
Richelle, Isabelle ULg

Conference (2014, January 23)

This conference will explore the history, impact, and potential for further development of the CJEU’s and the EFTA Court’s landmark decisions relating to direct taxation.

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See detailLost and found: Rediscovery of de Ryckholt's collection of Cretaceous Mollusca (Belgium and N. France)
Fischer, Valentin ULg; Denayer, Julien ULg; Mottequin, Bernard ULg

in 4th International Palaeontological Congress, Mendoza, Abstracts volume (2014)

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See detailLost Memories
Havelange, Carl ULg; DELRUE Rony

Book published by Hapax (2007)

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See detailLa loterie de l'Exposition (extrait d'une lettre adressée à M. Laisant)
Catalan, Eugène ULg

in Nouvelle Correspondance Mathématique (1879), 5

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See detailLoteries, combinatoire et heuristique
Breny, Henri; Esch, Louis ULg

in Mathématique et Pédagogie (1983), 40

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See detailLa Lotharingie, terre des utopies contemporaines
Robelin, Cyril ULg

in Les cahiers de l'Association de la Renaissance Lotharingienne (2013)

L'espace que nous appelons lotharingien, hérité de la partition de l'empire de Charlemagne, fut bien au XXe siècle la terre des utopies (au pluriel). Sans doute cette longue situation d'entre deux lui a ... [more ▼]

L'espace que nous appelons lotharingien, hérité de la partition de l'empire de Charlemagne, fut bien au XXe siècle la terre des utopies (au pluriel). Sans doute cette longue situation d'entre deux lui a donné cette inventivité de voir l'état autrement. A côté des deux grands colosses que sont la France et l'Allemagne (l'Empire), il a fallu sans cesse faire preuve de flexibilité pour exister. Mais ces XIXe et XXe siècles qui voient triompher le concept d'état-nation, sont aussi ceux des utopies. C'est dans cet espace où les frontières ont longtemps été contestées et changeantes, que les réalisations les plus brillantes ont vu le jour. C'est toujours dans un esprit de paix et de réconciliation que ces « Lotharingiens » vont choisir l'utopie pour résoudre les problèmes les plus épineux de l'Europe de l'Ouest. Bien souvent, ce sont les circonstances de l'époque qui font que l'on est obligé de penser autrement pour préserver la paix ou tout simplement pour exister et faire entendre sa voix [less ▲]

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See detailLotissement périurbain et développement durable : les surcoûts collectifs
Halleux, Jean-Marie ULg

Conference given outside the academic context (2001)

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See detailA LOTOS Data Facility Compiler (DAFY)
Lallemand, Eric; Leduc, Guy ULg

in Parther, Ken; Rose, Gordon (Eds.) Formal Description Techniques IV (1991, November)

If we take a look at existing LOTOS specifications, we notice that the description of the needed data types is very often huge. This causes the lack of concision of most descriptions of complex systems ... [more ▼]

If we take a look at existing LOTOS specifications, we notice that the description of the needed data types is very often huge. This causes the lack of concision of most descriptions of complex systems. We propose to tackle this problem in two steps. First, we define extensions to the LOTOS language allowing short definitions of most of the data types used in practical LOTOS specifications. Second, we propose a tool called `'DAFY'' (Data Facility Compiler) which is able to translate these extensions into standard LOTOS. [less ▲]

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See detailLOTOS, un outil utile ou un autre langage académique ?
Leduc, Guy ULg

in Danthine, André (Ed.) Les réseaux de communication - nouveaux outils et tendances actuelles (1987, January)

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See detailLOTUS: a low-cost, ultraviolet spectrograph
Steele, I. A.; Marchant, J. M.; Jermak, H. E. et al

in Monthly Notices of the Royal Astronomical Society (2016), 460

We describe the design, construction and commissioning of a simple, low-cost long-slit spectrograph for the Liverpool Telescope. The design is optimized for near-UV and visible wavelengths and uses all ... [more ▼]

We describe the design, construction and commissioning of a simple, low-cost long-slit spectrograph for the Liverpool Telescope. The design is optimized for near-UV and visible wavelengths and uses all transmitting optics. It exploits the instrument focal plane field curvature to partially correct axial chromatic aberration. A stepped slit provides narrow (2.5 × 95 arcsec) and wide (5 × 25 arcsec) options that are optimized for spectral resolution and flux calibration, respectively. On sky testing shows a wavelength range of 3200-6300 Å with a peak system throughput (including detector quantum efficiency) of 15 per cent and wavelength dependent spectral resolution of R = 225-430. By repeated observations of the symbiotic emission line star AG Peg, we demonstrate the wavelength stability of the system is <2 Å rms and is limited by the positioning of the object in the slit. The spectrograph is now in routine operation monitoring the activity of comet 67P/Churyumov-Gerasimenko during its current post-perihelion apparition. [less ▲]

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See detailLotze en Amérique. Le renouveau réaliste en philosophie chez Santayana
Boccaccini, Federico ULg

in Boccaccini, Federico (Ed.) Lotze et son héritage. Son influence et son impact sur la philosophie du XXème siècle (2015)

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See detailLotze en Amérique. Le renouveau réaliste en philosophie chez Santayana
Boccaccini, Federico ULg

Conference (2011, December)

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See detailLotze et la psychologie physiologique
Seron, Denis ULg

in Boccaccini, Federico (Ed.) Lotze et son héritage: Son influence et son impact sur la philosophie du XXe siècle (2015)

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See detailLotze et son héritage. Son influence et son impact sur la philosophie du XXème siècle
Boccaccini, Federico ULg

Book published by Peter Lang (2014)

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See detailLoud & Bright Purple Birds & Blue Eyed Bitches Dancing. A Task for Poetry # 2
Spinoy, Erik ULg

Book published by Onomatopee (2009)

The present volume is an introduction to the work of the young Belgian artist Tamara Van San. It contains two articles, an interview and a portfolio.

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See detailLouis Althusser. Politique et subjectivité. Editorial
Bruschi, Fabio; Mancuso, Eva ULg

in Cahiers du GRM (2015), VII

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See detailLouis Couperus: Eline Vere (study pack)
Steyaert, Kris ULg; Eagar, Erna

Learning material (2002)

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See detailLouis d’Orléans, le faste furieux d’un ambitieux
Marchandisse, Alain ULg

in Historia (2016), (835-836), 54-55

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See detailLouis Pasteur 1822-1895
Marchal, Émile ULg

in Bulletin de la Société Belge de Microscopie (1895), XXI