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See detailA novel mutation in the proteolipid protein gene leading to Pelizaeus-Merzbacher disease.
Otterbach, B.; Stoffel, W.; RAMAEKERS, Vincent ULg

in Biological chemistry Hoppe-Seyler (1993), 374(1), 75-83

Point mutations of the gene of human proteolipid protein (PLP) have been recognized as the molecular basis of one form of leukodystrophy, the X-chromosome-linked Pelizaeus-Merzbacher disease (PMD). We ... [more ▼]

Point mutations of the gene of human proteolipid protein (PLP) have been recognized as the molecular basis of one form of leukodystrophy, the X-chromosome-linked Pelizaeus-Merzbacher disease (PMD). We report the molecular analysis of four PMD patients in three unrelated families and describe a point mutation (G-->A transition) in exon V which leads to the substitution of Gly216 by a serine residue in a highly conserved extracytosolic domain and a Mae I RFLP. Molecular modelling with energy minimization indicates that this seemingly minor alteration of the amino-acid sequence induces a considerable conformational change and tight packing of the polypeptide chain apparently not compatible with the regular PLP function in oligodendrocytes. This mutation has been detected and characterized by PCR amplification of genomic DNA using intron and exon primers and the complete sequence analysis of the seven exons and a 300 bp promoter region of the PLP gene of two affected brothers. The sequence analysis of a PCR fragment representing exon V amplified from genomic DNA of different kindreds of the pedigree revealed the mother as the only carrier indicating that the mutation has occurred de novo in the mother's germline. PLP gene (including the 8.8 kb intron I) rearrangements have been excluded by Southern blot hybridization and overlapping PCR amplification of genomic DNA. [less ▲]

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See detailA novel mutation of the luteinizing hormone/choionic gonadotrophin receptor gene leading to Leydig cell hypoplasia type I
Potorac, Iulia ULg; Rivero-Müller, Adolfo; Pintiaux, Axelle ULg et al

in Abstract book - 24th Meeting of the Belgian Endocrine Society (2014, October 18)

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See detailA novel neurodevelopmental syndrome responsive to 5-hydroxytryptophan and carbidopa.
RAMAEKERS, Vincent ULg; Senderek, J.; Hausler, M. et al

in Molecular genetics and metabolism (2001), 73(2), 179-87

Tryptophan hydroxylase (TPH; EC 1.14.16.4) catalyzes the first rate-limiting step of serotonin biosynthesis by converting l-tryptophan to 5-hydroxytryptophan. Serotonin controls multiple vegetative ... [more ▼]

Tryptophan hydroxylase (TPH; EC 1.14.16.4) catalyzes the first rate-limiting step of serotonin biosynthesis by converting l-tryptophan to 5-hydroxytryptophan. Serotonin controls multiple vegetative functions and modulates sensory and alpha-motor neurons at the spinal level. We report on five boys with floppiness in infancy followed by motor delay, development of a hypotonic-ataxic syndrome, learning disability, and short attention span. Cerebrospinal fluid (CSF) analysis showed a 51 to 65% reduction of the serotonin end-metabolite 5-hydroxyindoleacetic acid (5HIAA) compared to age-matched median values. In one out of five patients a low CSF 5-methyltetrahydrofolate (MTHF) was present probably due to the common C677T heterozygous mutation of the methylenetetrahydrofolate reductase (MTHFR) gene. Baseline 24-h urinary excretion showed diminished 5HIAA values, not changing after a single oral load with l-tryptophan (50-70 mg/kg), but normalizing after 5-hydroxytryptophan administration (1 mg/kg). Treatment with 5-hydroxytryptophan (4-6 mg/kg) and carbidopa (0.5-1.0 mg/kg) resulted in clinical amelioration and normalization of 5HIAA levels in CSF and urine. In the patient with additional MTHFR heterozygosity, a heterozygous missense mutation within exon 6 (G529A) of the TPH gene caused an exchange of valine by isoleucine at codon 177 (V177I). This has been interpreted as a rare DNA variant because the pedigree analysis did not provide any genotype-phenotype correlation. In the other four patients the TPH gene analysis was normal. In conclusion, this new neurodevelopmental syndrome responsive to treatment with 5-hydroxytryptophan and carbidopa might result from an overall reduced capacity of serotonin production due to a TPH gene regulatory defect, unknown factors inactivating the TPH enzyme, or selective loss of serotonergic neurons. [less ▲]

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See detailNovel norovirus recombinants and GII.4 sub-lineages associated with outbreaks between 2006 and 2010 in Belgium
Mathijs, Elisabeth ULg; Denayer, Sarah; Palmeira, Leonor et al

in Virology Journal (2011)

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See detailA Novel Oxidizing Reagent Based on Potassium Ferrate(VI)
Delaude, Lionel ULg; Laszlo, Pierre ULg

in Journal of Organic Chemistry (1996), 61(18), 6360-6370

A new, efficient preparation has been devised for potassium ferrate(VI) (K(2)FeO(4)). The ability of this high-valent iron salt for oxidizing organic substrates in nonaqueous media was studied. Using ... [more ▼]

A new, efficient preparation has been devised for potassium ferrate(VI) (K(2)FeO(4)). The ability of this high-valent iron salt for oxidizing organic substrates in nonaqueous media was studied. Using benzyl alcohol as a model, the catalytic activity of a wide range of microporous adsorbents was ascertained. Among numerous solid supports of the aluminosilicate type, the K10 montmorillonite clay was found to be best at achieving quantitative formation of benzaldehyde, without any overoxidation to benzoic acid. The roles of the various parameters (reaction time and temperature, nature of the solvent, method of preparation of the solid reagent) were investigated. The evidence points to a polar reaction mechanism. The ensuing procedure was applied successfully, at room temperature, to oxidation of a series of alcohols to aldehydes and ketones, to oxidative coupling of thiols to disulfides, and to oxidation of nitrogen derivatives. At 75 degrees C, the reagent has the capability of oxidizing both activated and nonactivated hydrocarbons. Toluene is turned into benzyl alcohol (and benzaldehyde). Cycloalkanes are also oxidized, in significant (30-40%) yields, to the respective cycloalkanols (and cycloalkanones). Thus, potassium ferrate, used in conjunction with an appropriate heterogeneous catalyst, is a strong and environmentally friendly oxidant. [less ▲]

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See detailA novel pattern recognition approach to transient stability assessment of power systems
Belhomme, Régine; Van Cutsem, Thierry ULg; Pavella, Mania ULg

in Proc. 10th IFAC World Congress (1987)

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See detailNovel perspectives in the definition of responders in trials of osteoarthritis
Bruyère, Olivier ULg

Conference (2012, March 23)

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See detailNovel perspectives in the pathogenesis of type 1 diabetes
Geenen, Vincent ULg

Conference (2005, November)

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See detailNovel pH or thermosensitive block copolymers for triggered drug-delivery systems
Jérôme, Christine ULg

Conference (2007, October 30)

Over the last decade, polymer micelles and nanoparticles have attracted an increasing interest as efficient drug delivery systems. Polymer micelles from amphiphilic block copolymers are supramolecular ... [more ▼]

Over the last decade, polymer micelles and nanoparticles have attracted an increasing interest as efficient drug delivery systems. Polymer micelles from amphiphilic block copolymers are supramolecular core-shell type assemblies of some tens of nanometers in diameter. They are highly stable in aqueous solution because of their low intrinsic critical micelle concentration, which prevents their dissociation upon dilution in the blood stream after intravenous injection. The combination of poly(ethylene oxide) (PEO) with hydrophobic aliphatic polyesters, such as poly(ε-caprolactone) (PCL) or polylactide (PLA), allows to prepare stealthy drug nanocarriers (thanks to PEO), which are biodegradable and biocompatible and capable of encapsulating a hydrophobic drug (due to the aliphatic polyester). In this field, the inclusion of an additional pH-responsive block in the supramolecular assembly is a promising strategy to improve the targeting of tumor tissues by taking advantage of the lower pH at the vicinity of tumor cells. A variety of novel amphiphilic and pH-sensitive copolymers have been synthesized and tested as building blocks for the design of smart nanocarriers able to expose selectively the targeting unit at the vicinity of tumor. Various macromolecular architectures combining PEO, PCL and P2VP have been accordingly synthesized such as linear and star shape di- and triblock copolymers. Their (co)-micellization has been studied in terms of size, particle shape (DLS, TEM) and stealth behavior. Besides, thermo-responsive copolymers combining PEO, polyacrylic acid and poly-N-isopropyl-acrylamide have been synthesized and found efficient in stabilizing stealthy magnetic nanoparticles promising for thermally triggered release of drugs during hyperthermia. [less ▲]

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See detailNovel pH responsive-copolymers for smart drug carriers
Jérôme, Christine ULg

Conference (2008, September 22)

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See detailA novel pH sensitive water soluble fluorescent nanomicellar sensor for potential biomedical applications
Georgiev, N. I.; Bryaskova, Rayne; Tzoneva, R. et al

in Bioorganic & Medicinal Chemistry (2013), 21(21), 6292-6303

Herein we report on the synthesis and sensor activity of a novel pH sensitive probe designed as highly water-soluble fluorescent micelles by grafting of 1,8-naphthalimide–rhodamine bichromophoric FRET ... [more ▼]

Herein we report on the synthesis and sensor activity of a novel pH sensitive probe designed as highly water-soluble fluorescent micelles by grafting of 1,8-naphthalimide–rhodamine bichromophoric FRET system (RNI) to the PMMA block of a well-defined amphiphilic diblock copolymer—poly(methyl methacrylate)–b-poly(methacrylic acid) (PMMA48–b-PMAA27). The RNI-PMMA48–b-PMAA27 adduct is capable of self-assembling into micelles with a hydrophobic PMMA core, containing the anchored fluorescent probe, and a hydrophilic shell composed of PMAA block. Novel fluorescent micelles are able to serve as a highly sensitive pH probe in water and to internalize successfully HeLa and HEK cells. Furthermore, they showed cell specificity and significantly higher photostability than that of a pure organic dye label such as BODIPY. The valuable properties of the newly prepared fluorescent micelles indicate the high potential of the probe for future biological and biomedical applications. [less ▲]

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See detailNovel pH-responsive copolymers for smart drug carriers
Jérôme, Christine ULg

Conference (2009, November 18)

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See detailA novel phase portrait for neuronal excitability
Drion, Guillaume ULg; Franci, Alessio ULg; Seutin, Vincent ULg et al

in PLoS ONE (2012), 7(8),

Fifty years ago, FitzHugh introduced a phase portrait that became famous for a twofold reason: it captured in a physiological way the qualitative behavior of Hodgkin-Huxley model and it revealed the power ... [more ▼]

Fifty years ago, FitzHugh introduced a phase portrait that became famous for a twofold reason: it captured in a physiological way the qualitative behavior of Hodgkin-Huxley model and it revealed the power of simple dynamical models to unfold complex firing patterns. To date, in spite of the enormous progresses in qualitative and quantitative neural modeling, this phase portrait has remained a core picture of neuronal excitability. Yet, a major difference between the neurophysiology of 1961 and of 2011 is the recognition of the prominent role of calcium channels in firing mechanisms. We show that including this extra current in Hodgkin-Huxley dynamics leads to a revision of FitzHugh-Nagumo phase portrait that affects in a fundamental way the reduced modeling of neural excitability. The revisited model considerably enlarges the modeling power of the original one. In particular, it captures essential electrophysiological signatures that otherwise require non-physiological alteration or considerable complexification of the classical model. As a basic illustration, the new model is shown to highlight a core dynamical mechanism by which calcium channels control the two distinct firing modes of thalamocortical neurons. [less ▲]

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See detailNovel phenolic glycolipids: antioxidant activity and effect on membrane models
Sainvitu, Pauline ULg; Nasir, Mehmet Nail ULg; Draguet, Florian et al

Poster (2013, May 30)

Aromatic glycolipids are of both medical as well as pharmaceutical interest. Antimicrobial, antiviraland antiinflammatory activities have been reported (Otto, 2000, Journal of Molecular Catalysis B ... [more ▼]

Aromatic glycolipids are of both medical as well as pharmaceutical interest. Antimicrobial, antiviraland antiinflammatory activities have been reported (Otto, 2000, Journal of Molecular Catalysis B: Enzymatic). Moreover, they are expected to have interesting antioxidant properties when they contain phenolic groups. The alkyl chain should enhance their ability to penetrate into the cellular membrane (Nicolosi, 2002, Journal of Molecular Catalysis B: Enzymatic). The presence of a sugar unit could also be useful to target specific cells. In this study, novel aromatic glycolipids were synthesized as useful models for studying the structure–activity relationship, in particular as regards to their aromatic group.Theireffect on cell viability when an oxidative stress is induced was tested. In parallel, their interaction with cell models (liposomes) was studied through membrane fusion and permeability experiments. [less ▲]

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See detailNovel photobioreactors based on microalgae immobilized in silica gels obtained by "Chimie douce" for biofuel production
Léonard, Alexandre ULg; Rooke, J.; Sarmento, H. et al

in Abstracts of Papers - American Chemical Society, National Meeting (2010)

A new generation of photobioreactors for the clean production of green biofuels is described. It is based on the smart combination of living and inanimate, namely microalgae encapsulated in porous silica ... [more ▼]

A new generation of photobioreactors for the clean production of green biofuels is described. It is based on the smart combination of living and inanimate, namely microalgae encapsulated in porous silica gels. Such hybrid materials would allow for the controlled exploitation of photosynthesis to assimilate atmospheric CO2 and convert it into useful energy sources by using sunlight. The use of biocompatible routes for the immobilization of cyanobacteria has extended the duration of survival up to 40 weeks. [less ▲]

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See detailNovel photosynthetic CO2 bioconvertor based on green algae entrapped in low-sodium silica gels
Rooke, J. C.; Léonard, Alexandre ULg; Sarmento, H. et al

in Journal of Materials Chemistry (2011), 21(4), 951-959

A photosynthetic bioreactor for CO2 assimilation has been designed using silica sol-gel immobilisation technologies with the chlorophyta Botryococcus braunii (Kützing) and Chlorella vulgaris (Beijerinck ... [more ▼]

A photosynthetic bioreactor for CO2 assimilation has been designed using silica sol-gel immobilisation technologies with the chlorophyta Botryococcus braunii (Kützing) and Chlorella vulgaris (Beijerinck). The living hybrid gels formed revealed a mesoporosity that enabled diffusion of nutrients and gases, promoting the light and dark photosynthetic reactions from within the bulk of the material. To determine the efficiency of the photosynthetic bioreactor in terms of CO 2 remediation, the activity and viability of the encapsulated cells have been monitored through oximetry, 14C assimilation, pulse amplitude modulation fluorimetry and confocal microscopy, revealing a long term productivity of living hybrid materials capable of photosynthetic processes for at least 80 days. Structural and textural properties of the gels were established through 29Si MAS-NMR and N2 physisorption respectively. © 2011 The Royal Society of Chemistry. [less ▲]

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See detailNovel plasma extraction procedure and development of a specific enzyme immunoassay of oxytocin: application to biological and clinical investigations of small-cell carcinoma of the lung
Pequeux, Christel ULg; Hendrick, Jean-Claude; Hagelstein, Marie-Thérèse ULg et al

in Scandinavian Journal of Clinical & Laboratory Investigation (2001), 61(5), 407-415

Paraneoplastic secretion of the lactation-inducing hormone oxytocin (OT) has been reported in about 30% of cases of small cell carcinoma of the lung (SCCL). In order to investigate the role of OT in the ... [more ▼]

Paraneoplastic secretion of the lactation-inducing hormone oxytocin (OT) has been reported in about 30% of cases of small cell carcinoma of the lung (SCCL). In order to investigate the role of OT in the biology of SCCL tumours, a specific enzyme-immunoassay(EIA) for OT, which can be applied to both human plasma <br />and culturemedium, has been developed. OT EIA is performed on 96-well microtiter plates coated with a rabbit polyclonal antibody (Ab) anti-OT (O4). This antibody does not exhibit any significant cross-reactivity either with vasopressin (VP) or with vasotocin (VT). The immunological reaction involving Ab anti-OT is a competition between the tracer (biotinylatedOT) and syntheticOT (standard curve) or OT present in biological samples. In order to limit interference induced by plasma proteins, plasma samples are titrated by a one-step centrifugation on centricon YM-3 (cut-oOE 3000 Da). After plasma filtration, 90.7 ± 5.1 (SD) % (n = 22) immunoreactive ( IR) OT is recovered. The sensitivity of OT EIA is 1 pmol/L, while intra- and inter-assay coefficients of variation (CV) are around 3.41% and 2.84%, respectively. In healthy volunteers, plasma IR OT is 7.28 ± 4.49 (SD) pmol/L (n = 32) with no gender diOEerence. As shown by the data both from plasma of SCCL patients and from supernatants and cell contents of SCCL cell lines, this EIA procedure offers a novel, reproducible, specific and sensitive method for the measurement of IR OT. [less ▲]

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See detailNovel post-digest isotope coded protein labeling method for phospho- and glycoproteome analysis
Fleron, Maximilien ULg; Greffe, Yannick ULg; Musmeci, Davide ULg et al

in Journal of Proteomics (2010), 73(10), 1986-2005

In the field of proteomics there is an apparent lack of reliable methodology for quantification of posttranslational modifications. Present study offers a novel post-digest ICPL quantification strategy ... [more ▼]

In the field of proteomics there is an apparent lack of reliable methodology for quantification of posttranslational modifications. Present study offers a novel post-digest ICPL quantification strategy directed towards characterization of phosphorylated and glycosylated proteins. The value of the method is demonstrated based on the comparison of two prostate related metastatic cell lines originating from two distinct metastasis sites (PC3 and LNCaP). The method consists of protein digestion, ICPL labeling, mixing of the samples, PTM enrichment and MS-analysis. Phosphorylated peptides were isolated using TiO(2), whereas the enrichment of glycosylated peptides was performed using hydrazide based chemistry. Isolated PTM peptides were analyzed along with non enriched sample using 2D-(SCX-RP)-Nano-HPLC-MS/MS instrumentation. Taken together the novel ICPL labeling method offered a significant improvement of the number of identified (∼600 individual proteins) and quantified proteins (>95%) in comparison to the classical ICPL method. The results were validated using alternative protein quantification strategies as well as label-free MS quantification method. On the biological level, the comparison of PC3 and LNCaP cells has shown specific modulation of proteins implicated in the fundamental process related to metastasis dissemination. Finally, a preliminary study involving clinically relevant autopsy cases reiterated the potential biological value of the discovered proteins. [less ▲]

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See detailA novel potassium channel gene, KCNQ2, is mutated in an inherited epilepsy of newborns.
Singh, N. A.; Charlier, Carole ULg; Stauffer, D. et al

in Nature Genetics (1998), 18(1), 25-9

Idiopathic generalized epilepsies account for about 40% of epilepsy up to age 40 and commonly have a genetic basis. One type is benign familial neonatal convulsions (BFNC), a dominantly inherited disorder ... [more ▼]

Idiopathic generalized epilepsies account for about 40% of epilepsy up to age 40 and commonly have a genetic basis. One type is benign familial neonatal convulsions (BFNC), a dominantly inherited disorder of newborns. We have identified a sub-microscopic deletion of chromosome 20q13.3 that co-segregates with seizures in a BFNC family. Characterization of cDNAs spanning the deleted region identified one encoding a novel voltage-gated potassium channel, KCNQ2, which belongs to a new KQT-like class of potassium channels. Five other BFNC probands were shown to have KCNQ2 mutations, including two transmembrane missense mutations, two frameshifts and one splice-site mutation. This finding in BFNC provides additional evidence that defects in potassium channels are involved in the mammalian epilepsy phenotype. [less ▲]

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See detailA novel pyrid-3-yl sulfonylurea, BM 27, effective in brain edema and stroke treatment
Masereel, B.; Renard, P.; Pirotte, Bernard ULg et al

Conference (1994, September)

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