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See detailNeonatal progeroid variant of Marfan syndrome with congenital lipodystrophy results from mutations at the 3' end of FBN1 gene.
Jacquinet, Adeline ULg; Verloes, Alain; Callewaert, Bert et al

in European journal of medical genetics (2014), 57(5), 230-4

We report a 16-year-old girl with neonatal progeroid features and congenital lipodystrophy who was considered at birth as a possible variant of Wiedemann-Rautenstrauch syndrome. The emergence of ... [more ▼]

We report a 16-year-old girl with neonatal progeroid features and congenital lipodystrophy who was considered at birth as a possible variant of Wiedemann-Rautenstrauch syndrome. The emergence of additional clinical signs (marfanoid habitus, severe myopia and dilatation of the aortic bulb) lead to consider the diagnosis of the progeroid variant of Marfan syndrome. A de novo donor splice-site mutation (c.8226+1G>A) was identified in FBN1. We show that this mutation leads to exon 64 skipping and to the production of a stable mRNA that should allow synthesis of a truncated profibrillin-1, in which the C-terminal furin cleavage site is altered. FBN1 mutations associated with a similar phenotype have only been reported in four other patients. We confirm the correlation between marfanoid phenotype with congenital lipodystrophy and neonatal progeroid features (marfanoid-progeroid-lipodystrophy syndrome) and frameshift mutations at the 3' end of FBN1. This syndrome should be considered in differential diagnosis of neonatal progeroid syndromes. [less ▲]

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See detailThree arginine to cysteine substitutions in the pro-alpha (I)-collagen chain cause Ehlers-Danlos syndrome with a propensity to arterial rupture in early adulthood.
Malfait, Fransiska; Symoens, Sofie; De Backer, Julie et al

in Human Mutation (2007), 28(4), 387-95

Mutations in the COL1A1 and COL1A2 genes, encoding the proalpha1 and 2 chains of type I collagen, cause osteogenesis imperfecta (OI) or Ehlers-Danlos syndrome (EDS) arthrochalasis type. Although the ... [more ▼]

Mutations in the COL1A1 and COL1A2 genes, encoding the proalpha1 and 2 chains of type I collagen, cause osteogenesis imperfecta (OI) or Ehlers-Danlos syndrome (EDS) arthrochalasis type. Although the majority of missense mutations in the collagen type I triple helix affect glycine residues in the Gly-Xaa-Yaa repeat, few nonglycine substitutions have been reported. Two arginine-to-cysteine substitutions in the alpha1(I)-collagen chain are associated with classic EDS [R134C (p.R312C)] or autosomal dominant Caffey disease with mild EDS features [R836C (p.R1014C)]. Here we show alpha1(I) R-to-C substitutions in three unrelated patients who developed iliac or femoral dissection in early adulthood. In addition, manifestations of classic EDS in Patient 1 [c.1053C>T; R134C (p.R312C); X-position] or osteopenia in Patients 2 [c.1839C>T; R396C (p.R574C); Y-position] and 3 [c.3396C>T; R915C (p.R1093C); Y-position] are seen. Dermal fibroblasts from the patients produced disulfide-bonded alpha1(I)-dimers in approximately 20% of type I collagen, which were efficiently secreted into the medium in case of the R396C and R915C substitution. Theoretical stability calculations of the collagen type I heterotrimer and thermal denaturation curves of monomeric mutant alpha1(I)-collagen chains showed minor destabilization of the collagen helix. However, dimers were shown to be highly unstable. The R134C and R396C caused delayed procollagen processing by N-proteinase. Ultrastructural findings showed collagen fibrils with variable diameter and irregular interfibrillar spaces, suggesting disturbed collagen fibrillogenesis. Our findings demonstrate that R-to-C substitutions in the alpha1(I) chain may result in a phenotype with propensity to arterial rupture in early adulthood. This broadens the phenotypic range of nonglycine substitutions in collagen type I and has important implications for genetic counseling and follow-up of patients carrying this type of mutation. [less ▲]

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See detailIdentification of factors that maintain mammalian outer hair cells in adult organ of Corti explants
Malgrange, Brigitte ULg; Rigo, Jean-Michel; Coucke, Paul et al

in Hearing Research (2002), 170(1-2), 48-58

Both outer hair cells (OHCs) and inner hair cells (IHCs) survive and mature in 3 days old rat organ of Corti explants cultured for I month in a minimal essential medium. In contrast. under the same ... [more ▼]

Both outer hair cells (OHCs) and inner hair cells (IHCs) survive and mature in 3 days old rat organ of Corti explants cultured for I month in a minimal essential medium. In contrast. under the same culture conditions, only IHCs survive in explants from adult guinea pig organ of Corti while many of the OHCs are lost within the first 48 It. Hair cell Count,, show OHCs loss to be greater in the lower portion (i.e. middle turn) of the cochlea than Lit the apex. Terminal deoxynucleotidyl transferase-mediated dUTP nick end labelling (TUNEL) indicates that there is DNA damage in adult OHCs, within 8 h of explantation. Treatment of the adult organ of Corti explants with either actinomycin D (10(-7) M) or cycloheximide (10(-6) M) prevents most OHC losses . According to these results apoptosis may be the mechanism of OHC loss in adult organ of Corti explants, Stable membrane potentials recorded from the OHCs in both uncultured and actinomycin D-treated organ of Corti explants cultured for 72 h demonstrate the functional integrity of these hair cells. OHC losses in the adult guinea pig, organ of Corti cultures can also be prevented by treatment with several of the growth factors tested. i.e. acidic fibroblast growth factor (aFGF), insulin-like growth factor-1 (IGF-1), epidermal growth factor (EGF), transforming growth factor-beta1 (TGF-beta1). and glial cell-derived neurotrophic factor (GDNF). The results of this study suggest that growth factor therapy may be applicable to the treatment of some hearing disorders. (C) 2002 Elsevier Science B.V. All rights reserved. [less ▲]

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See detailCultured Oligodendrocyte Progenitors Derived from Cerebral Cortex Express a Glycine Receptor Which Is Pharmacologically Distinct from the Neuronal Isoform
Belachew, Shibeshih ULg; Rogister, Bernard ULg; Rigo, Jean-Michel et al

in European Journal of Neuroscience (1998), 10(11), 3556-64

Using the whole-cell patch-clamp technique, we demonstrate glycine-induced currents in oligosphere-derived oligodendrocyte progenitors cultured from newborn rats. Similar inward currents are also ... [more ▼]

Using the whole-cell patch-clamp technique, we demonstrate glycine-induced currents in oligosphere-derived oligodendrocyte progenitors cultured from newborn rats. Similar inward currents are also triggered by beta-alanine and taurine, two established glycine receptor agonists. In our recording conditions, glycine-gated currents in oligodendrocyte progenitors reverse about 0 mV and are reversibly inhibited by the glycine competitive antagonist strychnine, the Cl- channel blocker picrotoxinin and the non-competitive antagonist cyanotriphenylborate. The oligodendrocyte progenitors glycine receptor (GlyR) differs from the corresponding neuronal receptor: [3H]strychnine binding data and the strychnine inhibition curve of glycine-induced currents in oligodendrocyte progenitor cultures suggest the existence of two strychnine binding sites on the oligodendroglial GlyR. Using total RNA isolated from oligodendrocyte progenitors cultures, reverse transcription-polymerase chain reaction analysis of glycine receptor subunit expression shows the presence of alpha2 and beta subunits and immunocytochemical stainings confirm that this GlyR contains an alpha subunit which is not alpha1. The molecular structure of the oligodendroglial GlyR could be either homopentameric alpha2 or heteromeric alpha2beta but in both cases, the sequence of the alpha2 or beta subunits have to be different from the known neuronal sequences in order to explain, respectively, the cyanotriphenylborate (alpha2) and picrotoxinin (beta) sensitivities. This work thus demonstrates that GlyR are expressed by oligodendrocytes obtained not only from spinal cord but also from supraspinal structures. The pharmacological properties and presumably the molecular structure of oligodendroglial GlyR are original. The physiological meaning of the presence of such receptors on developing and mature oligodendrocytes remains unknown. [less ▲]

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See detailDevelopmental Regulation of Neuroligand-Induced Responses in Cultured Oligodendroglia
Belachew, Shibeshih ULg; Malgrange, Brigitte ULg; Rigo, Jean-Michel et al

in Neuroreport (1998), 9(6), 973-80

Using whole-cell patch-clamp techniques, we show that oligosphere-derived oligodendrocyte progenitor cells (OP) display GABA-, glutamate-, 5-HT-, glycine- and acetylcholine-gated inward currents. When OP ... [more ▼]

Using whole-cell patch-clamp techniques, we show that oligosphere-derived oligodendrocyte progenitor cells (OP) display GABA-, glutamate-, 5-HT-, glycine- and acetylcholine-gated inward currents. When OP differentiate into oligodendrocytes (ODC), the amplitude of peak currents elicited by saturating concentrations of these transmitters decreases except for 5-HT. Intracellular Ca2+ concentration changes induced by microperfusion of glutamate, 5-HT, TRH, met-enkephalin and substance P were monitored using a fluo-3-based calcium imaging system. When OP cells differentiate into ODC, a global decrease of the proportion of responding cells is observed. During type-2 astrocytes commitment, this proportion decreases for 5-HT, TRH- and metenkephalin stimulations whereas it remains constant for substance P and glutamate. These data demonstrate a development regulation of neurotransmitter- and neuropeptide-induced responses within the oligodendroglial lineage. [less ▲]

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See detailDiazepam-Insensitive Gabaa Receptors on Postnatal Spiral Ganglion Neurones in Culture
Malgrange, Brigitte ULg; Rigo, Jean-Michel; Lefebvre, Philippe ULg et al

in Neuroreport (1997), 8(3), 591-6

Using dissociated spiral ganglion cell cultures obtained from 3-day-old rat cochlea, we investigated the response of auditory neurones to gamma-aminobutyric acid (GABA) using patch-clamp techniques. In ... [more ▼]

Using dissociated spiral ganglion cell cultures obtained from 3-day-old rat cochlea, we investigated the response of auditory neurones to gamma-aminobutyric acid (GABA) using patch-clamp techniques. In our recording conditions, GABA elicited inward currents in > 95% of the neurones which reversed around 0 mV. Similar inward currents were measured using isoguvacin, a specific agonist of GABAA receptors. GABA-gated currents were reversibly inhibited by the channel blocker picrotoxin and the GABA competitive antagonist bicuculline. These functional GABAA receptors are characterized by an insensitivity to benzodiazepines and a relatively high sensitivity to beta-carbolines and barbiturates. These results show that the GABAA receptor pharmacological properties of spiral ganglion neurones are close to those of cerebellar granule cells. [less ▲]

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See detailBeta-Carbolines Induce Apoptotic Death of Cerebellar Granule Neurones in Culture
Malgrange, Brigitte ULg; Rigo, Jean-Marie ULg; Coucke, Paul et al

in Neuroreport (1996), 7(18), 3041-5

Apart from its role in fast inhibitory transmission, only neurotrophic effects have been reported following activation of the GABAA receptor. Here, we show that n-butyl-beta-carboline-3-carboxylate and n ... [more ▼]

Apart from its role in fast inhibitory transmission, only neurotrophic effects have been reported following activation of the GABAA receptor. Here, we show that n-butyl-beta-carboline-3-carboxylate and n-methyl-beta-carboline-3-carboxamide, which are negative allosteric modulators of the GABAA receptor acting at the benzodiazepine site, are neurotoxic for cerebellar granule neurones in culture. The beta-carboline-induced neuronal death is apoptotic since DNA internucleosomal fragmentation was induced and the neurotoxicity could be prevented by inhibitors of mRNA or protein synthesis. As GABA and benzodiazepine ligands (diazepam and Ro 15-1788) protect cerebellar granule cells against beta-carboline-induced toxicity, these data raise the possibility that the interaction between the beta-carbolines and the GABAA receptor is the triggering event leading to neuronal apoptosis. [less ▲]

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See detailAstroglia-released factor with negative allosteric modulatory properties at the GABA A receptor.
Rigo, Jean-Michel; Belachew, Shibeshih ULg; Coucke, Paul et al

in Biochemical Pharmacology (1996), 52(3), 465-473

We have previously shown, using whole-cell patch-clamp techniques, that astrocytes release a negative allosteric modulator of the gamma-aminobutyric acid type A receptor (GABAA receptor) with beta ... [more ▼]

We have previously shown, using whole-cell patch-clamp techniques, that astrocytes release a negative allosteric modulator of the gamma-aminobutyric acid type A receptor (GABAA receptor) with beta-carboline-like properties, thus, likely to act at the benzodiazepine site. Here, using patch-clamp and binding techniques, we confirm that the low-molecular-weight fraction of astroglia-conditioned medium (ACM lmf) contains a factor(s) that negatively modulates GABAA-receptor function. This factor, like beta-carbolines, enhances the specific binding of [35S]t-butyl bicyclophosphorothionate (TBPS) to adult rat cortical membranes in the presence of GABA. However, it fails to interact with various ligands of the benzodiazepine (BZD) site of the GABAA receptor ([3H]flunitrazepam, [3H]Ro 15-1788 and [3H]Ro 15-4513). The question of the actual binding site of the astroglia-derived factor on the GABAA receptor, thus, remains open and can be addressed only after the purification of the active molecule(s) of ACM Imf has been completed, and a labeled form of the endogenous ligand becomes available. Taken together, however, the data suggest that type 1 astrocytes are able to modulate the effects of the main inhibitory neurotransmission in the central nervous system. [less ▲]

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See detailMetalloproteinases and serine proteases activities in mixed spheroids of mouse B16 melanoma cells and fibroblasts
Coucke, Paul; Baramova, Eugenia; Leprince, Pierre ULg et al

in International Journal of Oncology (1994), 5

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See detailEffects of α-Hederin on cells cultured in vitro
Quetin-Leclercq, Joelle; Danloy, Sophie; Coucke, Paul et al

Poster (1993, June)

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See detailCytological effects of culture media conditioned B16 melanoma cells and 3T3 fibroblasts
Coucke, Paul; Siwek, B.; Gillet, Marie-Claire ULg et al

in Anticancer Research (1991), 11(2), 801-804

Cytotoxic soluble fractions (M.W.<1,000) were prepared from media conditioned by mixed cultures of 3T3 fibroblasts and B16 cells. The ultrastructural analyses of cells (B16 or 3T3) treated with these ... [more ▼]

Cytotoxic soluble fractions (M.W.<1,000) were prepared from media conditioned by mixed cultures of 3T3 fibroblasts and B16 cells. The ultrastructural analyses of cells (B16 or 3T3) treated with these fractions revealed in them mitochondria swelling, blebs, broken membranes and dead cells. [less ▲]

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See detailCytotoxic and mitogenic activities in culture media conditioned by mouse B16 melanoma cells and 3T3 fibroblasts
Siwek, Brigitte; Wauthy, Jacques ULg; Coucke, Paul et al

in Anticancer Research (1991), 11(2), 755-759

Cytotoxic (M.W.< 1000) and mitogenic (M.W.>10000) soluble factors are released into media conditioned by pure or mixed populations of mouse 3T3 fibroblasts and B16 melanoma cells cutivated in vitro. They ... [more ▼]

Cytotoxic (M.W.< 1000) and mitogenic (M.W.>10000) soluble factors are released into media conditioned by pure or mixed populations of mouse 3T3 fibroblasts and B16 melanoma cells cutivated in vitro. They are generally more active on B16 than on fibroblasts. [less ▲]

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See detailInfluence of laminin and fibroblasts upon colony formation by B16 melanoma cells
De Leval, Laurence; Leyh, Philippe; Grégoire, Dominique et al

Poster (1990, September)

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See detailCharacterization of tridimensional mixed cultures of mouse B16 melanoma cells and 3T3 fibroblasts
Coucke, Paul; Siwek, Brigitte; Munaut, Carine ULg et al

Conference (1990, September)

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See detailToxic and mitogenic activities of culture media conditioned by B16 melanoma cells
Coucke, Paul; Siwek, Brigitte; Gillet, Marie-Claire ULg et al

Conference (1990)

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See detailInfluence of laminin and fibroblasts upon colony formation in the mouse by B16 melanoma cell spheroids
de Leval, Laurence; Leyh, Philippe; Grégoire, Dominique et al

in Anticancer Research (1990), 10(5B), 1447

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See detailCooperativity between fibroblasts and melanoma cells during invasion
Siwek, Brigitte; Munaut, Carine ULg; De Pauw, Marie-Claire ULg et al

Conference (1989)

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