BBB syndrome and G syndrome were originally reported as distinct X-linked disorders. Clinical studies indicated that BBB and G syndromes were likely to represent variant expression of the same ...disorder, now referred to as "Opitz" GBBB syndrome. Several occurrences of male-to-male transmission in both syndromes led to the hypothesis that GBBB syndrome was a single autosomal dominant, sex influenced disorder, now tentatively mapped to 5p12-13. We report on a large pedigree in which GBBB syndrome appears to cosegregate with a pericentric inversion of the X chromosome inv(X)(p22.3q26). It indicates the possible existence of a true X-linked form of GBBB syndrome, which does not appear phenotypically different from its autosomal counterpart. The gene could map in the vicinity of the breakpoints, in Xp or Xq. The existence of two genes affecting a common pathogenetic pathway could explain the gender-dependent expressivity of GBBB phenotype.
is one of the most prevalent pathogens colonizing 50% of the world's population and causing gastritis and gastric cancer. Even with triple and quadruple antibiotic therapies,
shows increased ...prevalence of resistance to conventional antibiotics and treatment failure. Due to their pore-forming activity, antimicrobial peptides (AMP) are considered as a good alternative to conventional antibiotics, particularly in the case of resistant bacteria. In this study, temporin-SHa (a frog AMP) and its analogs obtained by Gly to Ala substitutions were tested against
. Results showed differences in the antibacterial activity and toxicity of the peptides in relation to the number and position of D-Ala substitution. Temporin-SHa and its analog NST1 were identified as the best molecules, both peptides being active on clinical resistant strains, killing 90-100% of bacteria in less than 1 h and showing low to no toxicity against human gastric cells and tissue. Importantly, the presence of gastric mucins did not prevent the antibacterial effect of temporin-SHa and NST1, NST1 being in addition resistant to pepsin. Taken together, our results demonstrated that temporin-SHa and its analog NST1 could be considered as potential candidates to treat
, particularly in the case of resistant strains.
Abstract The aim of this study was to evaluate the physicochemical properties and the apical dentin bond strength of the tricalcium silicate-based Biodentine in comparison to white MTA and zinc oxide ...eugenol-based cement (ZOE). Setting time and radiopacity were evaluated according to ISO 6876:2012 specification. Final setting time, compressive strength and pH were also assessed. Material’s bond strength to the apical root canal dentin was measured by the push-out assay. Data were analyzed by ANOVA and Tukey-Krammer post-hoc test. Biodentine presented the shortest initial (16.2±1.48 min) and final setting time (35.4±5.55 min). Radiopacity of Biodentine (2.79±0.27 mmAl) does not agree with ISO 6876:2012 specifications. On the other hand, Biodentine showed higher compressive strength after 21 days (37.22±5.27 MPa) and higher dentin bond strength (11.2±2.16 MPa) in comparison to white MTA (27.68±3.56 MPa for compressive strength and 2.98±0.64 MPa for bond strength) (p<0.05). Both MTA and Biodentine produced an alkaline environment (approximately pH 10) (p>0.05) compared to ZOE (pH 7). It may be concluded that Biodentine exhibited faster setting, higher long-term compressive strength and bond strength to the apical dentin than MTA and ZOE.
Resumo O objetivo deste estudo foi avaliar as propriedades físico-químicas e a resistência de união à dentina apical do cimento Biodentine em comparação ao MTA branco e cimento à base de óxido de zinco e eugenol (OZE). O tempo de presa e a radiopacidade foram avaliados de acordo com as especificações ISO 6876:2012. O tempo de presa final, a resistência à compressão e o pH também foram avaliados. A resistência de união dos materiais à dentina apical do canal radicular foi avaliada por meio do ensaio push-out. Dados foram analisados por ANOVA e teste complementar de Tukey-Krammer. Biodentine apresentou o menor tempo de presa inicial (16,2±1,48 min) e final (35,4±5,55 min). Os valores de radiopacidade do Biodentine (2,79±0,27 mmAl) não estão de acordo com as especificações ISO 6876:2012. Por outro lado, este material apresentou maior resistência à compressão após 21 dias (37,22±5,27 MPa) e maiores valores de adesão à dentina (11,2±2,16 MPa) em comparação ao MTA branco (27,68±3,56 MPa de resistência à compressão e 2,98±0,64 MPa de resistência de união) (p>0.05). Ambos os materiais produziram ambiente alcalino (aproximadamente 10) (p>0.05) em comparação ao OZE (pH 7). Pode-se concluir que o Biodentine demonstrou endurecimento mais rápido e apresentou maior resistência à compressão e resistência de união à dentina apical do que MTA e OZE.
Background: To assess the prevalence of negative body image and weight loss behaviour among children in primary and secondary school. Methods: Data were collected during the routine health ...assessment, in 10 767 children in the 6th grade (9-10 years) of primary school and the 2nd grade (13-14 years) of secondary school in Utrecht, a province in The Netherlands. Weight loss behaviour and body image were assessed during an interview and weight and height were measured. Results: A total of 7.8% of the boys and 13.9% of the girls of primary school had a negative body image (P < 0.001); 2.9% of the boys and 6.9% girls found themselves too fat, while having a normal body weight (P < 0.001). Weight-loss behaviour is found in 3.7% of the boys and 7.0% of the girls (P < 0.001). At secondary school, 15.8% of the boys and 32.5% of the girls found themselves too fat (P < 0.001). A total of 8.6% of the boys and 27.5% of the girls found themselves too fat, while having a normal body weight (P < 0.001); 4.7% of the boys and 12.9% of the girls with a normal weight showed weight loss behaviour (P < 0.001). Conclusion: A negative body image and weight loss behaviour were already present in 9- to 10-year-old children and among children with a normal weight in The Netherlands. Among secondary school children (13-14 year), the prevalence of a negative body image and of weight loss behaviour was high, especially for girls. Diagnostic tools are needed for youth health-care workers to detect unnecessary weight loss behaviour.
A variety of drugs targeting monoamine receptors are routinely used in human pharmacology. We assessed the effect of these drugs on the viability of tumor‐initiating cells isolated from patients with ...glioblastoma. Among the drugs targeting monoamine receptors, we identified prazosin, an α1‐ and α2B‐adrenergic receptor antagonist, as the most potent inducer of patient‐derived glioblastoma‐initiating cell death. Prazosin triggered apoptosis of glioblastoma‐initiating cells and of their differentiated progeny, inhibited glioblastoma growth in orthotopic xenografts of patient‐derived glioblastoma‐initiating cells, and increased survival of glioblastoma‐bearing mice. We found that prazosin acted in glioblastoma‐initiating cells independently from adrenergic receptors. Its off‐target activity occurred via a PKCδ‐dependent inhibition of the AKT pathway, which resulted in caspase‐3 activation. Blockade of PKCδ activation prevented all molecular changes observed in prazosin‐treated glioblastoma‐initiating cells, as well as prazosin‐induced apoptosis. Based on these data, we conclude that prazosin, an FDA‐approved drug for the control of hypertension, inhibits glioblastoma growth through a PKCδ‐dependent mechanism. These findings open up promising prospects for the use of prazosin as an adjuvant therapy for glioblastoma patients.
Synopsis
Prazosin, an FDA‐approved drug for hypertension with a record of over 40 years of safe and effective clinical use, curbs intracranial glioblastoma growth in mice in a preclinical setting and is thus as a potential anti‐glioblastoma adjuvant drug.
Prazosin induces glioblastoma cell apoptosis.
PKCδ‐dependent inhibition of AKT pathway is identified as a possible mechanism for prazosin‐induced glioblastoma apoptosis, independently of adrenergic receptors.
Glioblastoma growth in orthotopic xenograft mouse models was inhibited by prazosin and, as a consequence, mice survival increased.
Prazosin, an FDA‐approved drug for hypertension with a record of over 40 years of safe and effective clinical use, curbs intracranial glioblastoma growth in mice in a preclinical setting and is thus as a potential anti‐glioblastoma adjuvant drug.
A variety of drugs targeting monoamine receptors are routinely used in human pharmacology. We assessed the effect of these drugs on the viability of tumor-initiating cells isolated from patients with ...glioblastoma. Among the drugs targeting monoamine receptors, we identified prazosin, an a1-and a2B-adrenergic receptor antagonist, as the most potent inducer of patient-derived glioblastoma-initiating cell death. Prazosin triggered apoptosis of glioblastoma-initiating cells and of their differentiated progeny, inhibited glioblastoma growth in orthotopic xenografts of patient-derived glioblastoma-initiating cells, and increased survival of glioblastoma-bearing mice. We found that prazosin acted in glioblastoma-initiating cells independently from adrenergic receptors. Its off-target activity occurred via a PKCd-dependent inhibition of the AKT pathway, which resulted in caspase-3 activation. Blockade of PKCd activation prevented all molecular changes observed in prazosin-treated glioblastoma-initiating cells, as well as prazosin-induced apoptosis. Based on these data, we conclude that prazosin, an FDA-approved drug for the control of hyperten-sion, inhibits glioblastoma growth through a PKCd-dependent mechanism. These findings open up promising prospects for the use of prazosin as an adjuvant therapy for glioblastoma patients.
The aim of this study was to evaluate the physicochemical properties and the apical dentin bond strength of the tricalcium silicate-based Biodentine in comparison to white MTA and zinc oxide ...eugenol-based cement (ZOE). Setting time and radiopacity were evaluated according to ISO 6876:2012 specification. Final setting time, compressive strength and pH were also assessed. Material's bond strength to the apical root canal dentin was measured by the push-out assay. Data were analyzed by ANOVA and Tukey-Krammer post-hoc test. Biodentine presented the shortest initial (16.2±1.48 min) and final setting time (35.4±5.55 min). Radiopacity of Biodentine (2.79±0.27 mmAl) does not agree with ISO 6876:2012 specifications. On the other hand, Biodentine showed higher compressive strength after 21 days (37.22±5.27 MPa) and higher dentin bond strength (11.2±2.16 MPa) in comparison to white MTA (27.68±3.56 MPa for compressive strength and 2.98±0.64 MPa for bond strength) (p<0.05). Both MTA and Biodentine produced an alkaline environment (approximately pH 10) (p>0.05) compared to ZOE (pH 7). It may be concluded that Biodentine exhibited faster setting, higher long-term compressive strength and bond strength to the apical dentin than MTA and ZOE.
Following the identification of a
M. tuberculosis phosphate transporter belonging to the superfamily of ABC transporters, we report on the cloning and sequencing of two additional genes, called
...pstS-3 and
pstC-2, encoding proteins homologous to PstS and PstC of
Escherichia coli, respectively. Together with the previously isolated
M. tuberculosis gene similar to the
E. coli pstA, these are included in a cluster encoding a second putative phosphate transport system. We demonstrate that
pstS-3 encodes the previously described Ag 88, a 40 kDa
M. bovis BCG culture filtrate antigen (immunodominant in H-2
b haplotype type mice). Finally, a signature motif identifying integral transmembrane proteins of prokaryotic phosphate binding-dependent permeases is proposed.
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