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Although the molecular mechanisms underlying congenital heart disease (CHD) remain poorly understood, recent advances in genetic analysis have facilitated the exploration of causative genes for CHD. We reported that the pathogenic variant c.1617del of TMEM260, which encodes a transmembrane protein, is highly associated with CHD, specifically persistent truncus arteriosus (PTA), the most severe cardiac outflow tract (OFT) defect. Using whole-exome sequencing, the c.1617del variant was identified in two siblings with PTA in a Japanese family and in three of the 26 DNAs obtained from Japanese individuals with PTA. The c.1617del of TMEM260 has been found only in East Asians, especially Japanese and Korean populations, and the frequency of this variant in PTA is estimated to be next to that of the 22q11.2 deletion, the most well-known genetic cause of PTA. Phenotype of patients with c.1617del appears to be predominantly in the heart, although TMEM260 is responsible for structural heart defects and renal anomalies syndrome (SHDRA). The mouse TMEM260 variant (p.W535Cfs*56), synonymous with the human variant (p.W539Cfs*9), exhibited truncation and downregulation by western blotting, and aggregation by immunocytochemistry. In situ hybridization demonstrated that Tmem260 is expressed ubiquitously during embryogenesis, including in the development of cardiac OFT implicated in PTA. This expression may be regulated by a ~ 0.8 kb genomic region in intron 3 of Tmem260 that includes multiple highly conserved binding sites for essential cardiac transcription factors, thus revealing that the c.1617del variant of TMEM260 is the major single-gene variant responsible for PTA in the Japanese population.
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Cardiopatias Congênitas , Proteínas de Membrana , Animais , Feminino , Humanos , Masculino , Camundongos , Povo Asiático/genética , População do Leste Asiático , Sequenciamento do Exoma , Predisposição Genética para Doença , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/patologia , Japão , Proteínas de Membrana/genética , Linhagem , FenótipoRESUMO
Viscoelastic relaxation mechanisms of individualized cellulose nanofibers (iCNFs) dispersed in glycerol in the dilute and semidilute regions were investigated by linear viscoelastic and dynamic birefringence measurements. The birefringence relaxation of the iCNFs was described by the orientational and curvature modes of an existing viscoelastic theory for ideal semiflexible polymers (Shankar-Pasquali-Morse theory). However, the Shankar-Pasquali-Morse theory could not fully describe the iCNF viscoelastic relaxation at high frequencies. Considering the results for birefringence relaxation, the experimental tension mode of the iCNFs was evaluated to be higher than the theoretical value. These results show that the viscoelastic relaxations of the iCNFs are different from those of ideal semiflexible polymers, in contrast to cellulose nanocrystals (CNCs). As the iCNF concentration increased, the orientational mode dramatically slowed, which was more drastic than other semiflexible polymers, including CNCs. This anomalous behavior is likely due to the nonideal nature of iCNFs.
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BACKGROUND: Limited healing potential of the meniscus remains a burden for the successful repair of meniscus injuries in the orthopaedic fields. Silk-elastin (SE) is a novel recombinant protein with favorable properties for wound healing. This proof-of-concept study aimed to investigate the therapeutic effect of silk-elastin in a rabbit meniscal defect model. METHODS: A migration assay using rabbit meniscus and synovial cells with various concentrations of SE in a culture medium was conducted to investigate the mechanism of meniscal healing by SE. Additionally, cylindrical defects with a 1.5 mm diameter were created at the anterior horn of the medial meniscus of rabbits. The animals were divided into three groups: 1) the Blank group; defect only, 2) the Col I group; implantation of type I atelocollagen sponge, and 3) the SE group; implantation of SE (150 mg/ml) sponge. Whole medial menisci were harvested at 4, 8, 12, and 24 weeks after surgery. Histological analyses including immunohistochemical staining were performed to assess meniscal healing. RESULTS: In vitro study, Migration assay demonstrated a significantly higher number of migrated cells only in synovial cells. Especially, the SE concentration of 10 µg/mL demonstrated the highest number of migrated cells compared with other concentrations. In vivo study, the SE group exhibited significantly higher Ishida scores than other groups at all time points. Furthermore, the SE group showed higher synovial coverage scores than the Col I group at 4 and 8 weeks. Immunohistochemical staining demonstrated higher type II collagen staining in the SE group compared to other groups at 12 weeks. Implanted SE was efficiently replaced by safranin-O staining positive tissue within 8 weeks. CONCLUSIONS: SE could effectively repair a meniscal defect by inducing coverage of synovial cells. SE has the potential to be a useful material for meniscal repair.
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Modelos Animais de Doenças , Seda , Lesões do Menisco Tibial , Cicatrização , Animais , Coelhos , Lesões do Menisco Tibial/cirurgia , Cicatrização/efeitos dos fármacos , Meniscos Tibiais/cirurgia , Movimento Celular , Estudo de Prova de Conceito , Masculino , Células CultivadasRESUMO
Vaginal cuff dehiscence after total hysterectomy or total cystectomy had been increasing since laparoscopic or robotic surgery became a common surgery among gynecologists and urologists. A 52-yearold woman underwent laparoscopic radical total cystectomy for muscle invasive bladder carcinoma at Rakuwakai Otowa Hospital. She was emergently admitted with a fist-sized lump protruding from her vagina four months after surgery. Physical examination and her past history on admission disclosed vaginal cuff dehiscence after cystectomy. Computed tomographic scan and magnetic resonance imaging showed no bowel evisceration in the lump. We confirmed that the content of lump was peritoneal tissue and removed it by laparoscopic surgery. Simultaneously, we repaired the vaginal cuff dehiscence with a gracilis myocutaneous flap. There was no subsequent recurrence of vaginal dehiscence or bladder carcinoma in one-year follow-up.
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Cistectomia , Laparoscopia , Neoplasias da Bexiga Urinária , Humanos , Feminino , Cistectomia/efeitos adversos , Pessoa de Meia-Idade , Neoplasias da Bexiga Urinária/cirurgia , Deiscência da Ferida Operatória/etiologia , Retalho Miocutâneo , Vagina/cirurgia , Complicações Pós-OperatóriasRESUMO
BACKGROUND: Accurate evaluation of hip instability is critical for the diagnosis and successful treatment of developmental dysplasia of the hip (DDH). However, dynamic evaluation of hip instability is not well established. This study aimed to use the lateral view from a radiograph to evaluate dynamic antero-posterior hip instability in patients with DDH. METHODS: Seventy-four patients (92 hips) with DDH (DDH group) and 46 patients (59 hips) without hip pain and DDH (Control group) were examined. A false profile view (FPV) according to Lequesne was taken at standard and 90° flexion with the hip of interest defined as functional FPV; the translation of the center of the femoral head (CFH) obtained from the functional FPV was measured. As a validation test, we measured the anterior translation of the CFH using ultrasonography (US). RESULTS: There was a significant difference between the two groups in the translation of the CFH (p < 0.01). The degree of CFH translation depended on the severity of DDH (lateral center edge angle, r = -0.56, p < 0.01; vertical center anterior margin angle, r = -0.57, p<0.01) and lateralization of the femoral head (head lateralization index, r = 0.54, p < 0.01). There was a significant correlation between functional FPV and US measurements (r = 0.71, p < 0.01). CONCLUSION: The present study confirmed that antero-posterior hip instability in DDH patients can be detected using functional FPV. Our novel measurement, as a new method for assessing hip instability, may be useful for evaluating hip dynamic instability in diagnosing the etiology, and determining and evaluating the treatment for DDH at lower cost and improved accessibility.
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Luxação Congênita de Quadril , Humanos , Luxação Congênita de Quadril/diagnóstico por imagem , Articulação do Quadril/diagnóstico por imagem , Radiografia , Artralgia , Cabeça do Fêmur/diagnóstico por imagem , Estudos Retrospectivos , AcetábuloRESUMO
BACKGROUND: To evaluate the relationships among hip instability, pain, and morphology of the iliofemoral ligament (ILFL) in patients with developmental dysplasia of the hip (DDH) using ultrasonography (US). METHODS: We reviewed 86 patients (109 hips) with DDH (Group D), 40 patients (46 hips) with borderline hip dysplasia (BDDH) (Group B) and 20 patients (23 hips) without hip pain and bony abnormality (control group). Group D was classified into three subgroups-the severe (group SP), moderate (group MP), and none/mild (group NMP) hip pain groups-using the visual analogue scale (VAS). For evaluating hip instability and ILFL morphology, the distance between the anterior edge of the anterior inferior iliac spine (AIIS) and the horizontal line to the femoral head, and ILFL thickness were measured using US. The difference between the distance in the neutral position and Patrick position was calculated and defined as the femoral head translation distance (FTD). RESULTS: FTD and ILFL thickness in group D were significantly larger than those in the control group and group B (P < 0.05). There was a significant positive correlation between FTD and ILFL thickness in three groups (r = 0.57, P < 0.05; r = 0.55, P < 0.05; r = 0.62, P < 0.05, respectively). FTD and ILFL thickness in group SP were significantly larger than those in group NMP (P < 0.05). FTD and ILFL thickness in group D had significantly negative correlations with the lateral center edge (r = -0.54, P < 0.05; r = -0.40, P < 0.05, respectively) and vertical-center-anterior angle (r = -0.51, P < 0.05; r = -0.43, P < 0.05, respectively). CONCLUSIONS: Acetabular bony deficiency, especially in the anterior and lateral region can result in antero-posterior hip instability, leading to thickened ILFL and hip pain, even in patients with BDDH. These findings may facilitate our understanding and treatment of patients with DDH. When hip instability is suspected, hip US examination may help confirm the diagnosis and assist in providing objective clinical diagnostic evidence.
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OBJECTIVES: To analyse the microstructural changes of subchondral trabecular bone in patients with osteonecrosis of the femoral head (ONFH) using multi-detector row computed tomography (MDCT). METHODS: We retrospectively investigated 76 hips in 50 patients diagnosed with ONFH between 2017 and 2021. Groups 1, 2, 3, and 4 comprised hips without ONFH, ONFH without femoral head collapse (FHC), ONFH with mild collapse (<2 mm), and ONFH with severe collapse (>2 mm), respectively. All patients underwent MDCT, and the subchondral trabecular bone microstructure was assessed. Regions of interests were set at the lateral boundary of the femoral head necrotic lesion and centre of the acetabular weight-bearing portion. RESULTS: In both the femoral head and the acetabular regions, there were significant differences in Groups 2 and 3 compared to Group 1, with increased volumetric bone mineral density and apparent bone volume fraction, and more plate-like with increased connectivity, indicating that osteosclerotic changes were occurring. CONCLUSIONS: In both the femoral head and the acetabular regions, osteosclerotic changes of subchondral trabecular bone microstructure were present before FHC.
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Osso Esponjoso , Necrose da Cabeça do Fêmur , Humanos , Estudos Retrospectivos , Osso Esponjoso/patologia , Cabeça do Fêmur , TomografiaRESUMO
Reversible cross-links by non-covalent bonds have been widely used to produce supramolecular hydrogels that are both tough and functional. While various supramolecular hydrogels with several kinds of reversible cross-links have been designed for many years, a universal design that would allow control of mechanical and functional properties remains unavailable. The physical properties of reversible cross-links are usually quantified by thermodynamics, dynamics, and bond energies. Herein, we investigated the relationship between the molecular mobility and mechanical toughness of supramolecular hydrogels consisting of two kinetically distinct reversible cross-links via host-guest interactions. The molecular mobility was quantified as the second-order average relaxation time (ãτãw) of the reversible cross-links. We discovered that hydrogels combining fast (ãτãw = 1.8 or 18 s) and slowly (ãτãw = 6.6 × 103 or 9.5 × 103 s) reversible cross-links showed increased toughness compared to hydrogels with only one type of cross-link because relaxation processes in the former occurred with wide timescales.
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Phase equilibrium and dielectric relaxation were examined for mixtures of liquid-crystalline (LC)-forming 4-cyano-4'-pentylbiphenyl (5CB) with dilute dimethyl phthalate (DMP). The mixtures were in an isotropic one-phase state at high temperatures T but were separated into nematic and isotropic phases at low T < TIN (isotropic-to-nematic transition temperature), and the isotropic phase disappeared and a nematic one-phase state was realized on a further decrease of T below another transition temperature . These TIN and data (phase diagram) were described considerably well by a simple model of free energy contributed from a Flory-Huggins type mixing entropy (no enthalpic contribution) and a Landau-de Gennes type nematic interaction. This success of the model, not expected for ordinary (not LC-forming) components exhibiting enthalpically-driven phase separation, suggested that the phase separation in the DMP/5CB mixtures was triggered by the nematic transition of 5CB (possibly governed by the packing entropy) and thus the orientation fluctuation of 5CB molecules was coupled with the composition fluctuation. This coupling was expected to affect the dielectric relaxation detecting the rotational dynamics of 5CB molecules, the major component in the mixtures. This expectation was examined for a representative mixture having the DMP content of wDMP = 3.1 wt% and TIN â 27.0 °C. In a high-T asymptote (T > TIN + 10 °C), the dielectric relaxation of this mixture was close to that of pure 5CB, which suggested no significant effect of the above coupling on 5CB dynamics in the mixture at such high T. Nevertheless, in a significantly wide range of T between TIN and TIN + 10 °C, the dielectric relaxation time τε of the isotropic one-phase mixture increased on cooling much more significantly compared to τε in that high-T asymptote. The kinematic viscosity ν of the mixture exhibited a qualitatively similar increase in the same range of T, but this increase was weaker than that of τε. This difference between the dielectric τε and the rheological ν was attributed to coupling of the orientation and the composition fluctuations mentioned above. Namely, the composition fluctuation enhances the orientation fluctuation in the mixture thereby providing τε (reflecting the orientation fluctuation) with an extra increase. Pure 5CB exhibited similar increases of τε and ν (stronger for the former) but only in a close vicinity of (within 2-3 °C), because the orientation fluctuation in pure 5CB is coupled with the density fluctuation, not with the composition fluctuation being absent in the pure 5CB system. This behavior of pure 5CB in turn suggests an importance of the coupling of orientation and composition fluctuations in the mixture.
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We report the viscoelastic relaxation mechanisms of cellulose nanocrystals (CNCs) dispersed individually in fluids. Linear viscoelasticity and flow birefringence of softwood and tunicate CNC/glycerol dispersions in the dilute regime were measured. The obtained results were then compared with molecular theories for the linear viscoelasticity of rigid rods and semiflexible rods at infinite dilution by taking length distributions of CNCs into account. Although CNCs are traditionally regarded as rigid Brownian rods, the viscoelastic relaxation was not explained solely by the rotational motions of rods. Alternatively, molecular theories for semiflexible rods well-described the viscoelastic behavior; the CNCs showed additional relaxation modes derived from microscopic internal motions including "tension" and "curvature", which originated from the finite flexural rigidity. Birefringence relaxation of the CNCs was dominated by the rotational motions of rods. Length distribution functions of the CNCs were thus calculated from the birefringence relaxation and agreed well with their microscopy-determined histograms.
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Celulose/química , Nanopartículas/química , Birrefringência , Glicerol/química , Temperatura , Fatores de Tempo , ViscosidadeRESUMO
BACKGROUND: Transcatheter closure of patent ductus arteriosus (TC-PDA), conventionally guided by aortography, has become the standard treatment of this disease. The purposes of this study were to evaluate whether intracardiac echocardiography (ICE) may be used for measuring PDA size and be used as a guide for TC-PDA. METHODS: This study had 2 phases. In phase 1, we compared the measurements of PDA size: pulmonary artery side diameter (PA-D), length, and aortic side diameter (Ao-D) of PDA, as measured by ICE with those measured by aortography or cardiac computed tomography (AoG/CCT) in 23 patients who underwent TC-PDA. In phase 2, we compared the demographics, fluoroscopic time, contrast volume, and complications of the TC-PDAs between 10 adult patients with ICE guidance and 16 without it. RESULTS: In phase 1, we found great correlation and agreement between ICE and AoG/CCT in PA-D (r = 0.985, bias -0.077 to 0.224), but moderate to poor correlation and agreement in length (r = 0.653, bias -0.491 to 3.065) and Ao-D (r = 0.704, bias 0.738 to 4.732), respectively. Nevertheless, all patients underwent successful TC-PDA with ICE guidance that allowed us to continuously monitor the whole process. In phase 2, TC-PDA required a significantly lower contrast volume with ICE guidance than without it, and there was no significant difference in the remaining variables between the 2 groups. CONCLUSION: ICE is comparable to AoG/CCT in providing accurate PA-D of the PDA and may be a safe alternative to guide TC-PDA as compared to conventional aortography.
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Cateterismo Cardíaco , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/cirurgia , Ecocardiografia , Ultrassonografia de Intervenção , Adulto , Aortografia , Pré-Escolar , Feminino , Fluoroscopia , Humanos , Lactente , Masculino , Artéria Pulmonar , Resultado do TratamentoRESUMO
BACKGROUND: Fibrillin-1, tropoelastin, fibulin-5, and latent transforming growth factor beta-binding protein-2 and protein-4 (LTBP-2 and LTBP-4) are essential proteins for the elastic lamina (EL). In this study, we analyzed each of these molecules in the EL of Bruch's membrane (BM) through development and aging. METHODS: C57BL/6 mice (embryonic (E) days E12.5, E15.5, and E18.5; postnatal (P) days P1, P4, and P7 and P3, P6, and P75 weeks of age) were used. To investigate localization, immunohistochemical staining (IH) was performed. Transmission electron microscopy (TEM) was used to evaluate the formation of microfibrils and tropoelastin. mRNA expression was determined by quantitative real-time PCR (qRT-PCR). RESULTS: All five proteins were expressed in the EL of BM by IH except in embryonic mice. TEM results showed that tropoelastin co-stained with microfibrils. Between 3 and 6 weeks of age, microfibrils became longer and thicker. It was difficult to evaluate the EL of BM in senile mice at 75 weeks of age because of abundant deposits which correspond to drusen. mRNA levels of each protein increased dramatically from E15.5 to P1 days and plateaued by P3 weeks as shown by qRT-PCR. CONCLUSIONS: In conclusion, these five proteins are possibly involved in elastic fiber assembly in BM. We define the date of full assembly of the EL of BM as 3 weeks of age in mice.
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Envelhecimento , Lâmina Basilar da Corioide/crescimento & desenvolvimento , Regulação da Expressão Gênica no Desenvolvimento , Proteínas dos Microfilamentos/genética , Prenhez , RNA Mensageiro/genética , Animais , Animais Recém-Nascidos , Lâmina Basilar da Corioide/metabolismo , Lâmina Basilar da Corioide/ultraestrutura , Feminino , Fibroblastos/metabolismo , Fibroblastos/ultraestrutura , Imuno-Histoquímica , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Microfibrilas/metabolismo , Microfibrilas/ultraestrutura , Proteínas dos Microfilamentos/biossíntese , Microscopia Eletrônica de Transmissão , Gravidez , RNA Mensageiro/biossíntese , Reação em Cadeia da Polimerase em Tempo RealRESUMO
Although DNA can form triplex and quadruplex structures through hydrogen bonds, design and preparation of structures with more than five strands is difficult even when artificial nucleic acids are used. Herein we report a hexaplex formed by oligomers of artificial nucleic acids bearing bifacial molecules on d-threoninol. Aminopyrimidine and cyanuric acid derivatives were selected as bases because they have complementary hydrogen bonding patterns. The complex formed by aminopyrimidine and cyanuric acid decamers melted with large hysteresis. Hexaplex formation was indicated by gel electrophoresis, size exclusion chromatography and atomic force microscopy imaging, and proven directly through native mass spectrometry. CD measurements and molecular dynamics simulations indicated that the hexaplex adopts a helical structure. The hexaplex formation was highly dependent on pH and the presence of divalent cations. The hexaplex was stable in aqueous solution, and its unique structure and properties may lead to novel nanostructures, molecular assemblies, metal sensors, and ion channels.
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Relationship between segmental relaxation and normal-mode relaxation has been studied for molten poly(butylene oxide)s having various molecular weights by broadband dielectric spectroscopy over a wide temperature (T) range. We found that T dependence of the segmental relaxation time, τs, was weaker than the normal mode time, τn, at high T(>250 K â¼ Tg + 50 K), and the τn/τs ratio systematically decreased with increasing temperature. This high temperature complexity, whose mechanism has not been discussed in detail so far, was quantitatively explained by assuming the two step processes: local conformation change of polymers (elementary process) occurs first, and then the motion of a segment unit (second process) occurs. It was also found that the elementary process was strongly correlated with the experimentally observed ß-relaxation.
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Latent TGF-ß-binding protein-2 (LTBP-2) is an extracellular matrix protein associated with microfibrils. Homozygous mutations in LTBP2 have been found in humans with genetic eye diseases such as congenital glaucoma and microspherophakia, indicating a critical role of the protein in eye development, although the function of LTBP-2 in vivo has not been well understood. In this study, we explore the in vivo function of LTBP-2 by generating Ltbp2(-/-) mice. Ltbp2(-/-) mice survived to adulthood but developed lens luxation caused by compromised ciliary zonule formation without a typical phenotype related to glaucoma, suggesting that LTBP-2 deficiency primarily causes lens dislocation but not glaucoma. The suppression of LTBP2 expression in cultured human ciliary epithelial cells by siRNA disrupted the formation of the microfibril meshwork by the cells. Supplementation of recombinant LTBP-2 in culture medium not only rescued the microfibril meshwork formation in LTBP2-suppressed ciliary epithelial cells but also restored unfragmented and bundled ciliary zonules in Ltbp2(-/-) mouse eyes under organ culture. Although several reported human mutant LTBP-2 proteins retain normal domain structure and keep the fibrillin-1-binding site intact, none of these mutant proteins were secreted from their producing cells, suggesting secretion arrest occurred to the LTBP-2 mutants owing to conformational alteration. The findings of this study suggest that LTBP-2 is an essential component for the formation of microfibril bundles in ciliary zonules.
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Cílios/genética , Proteínas de Ligação a TGF-beta Latente/genética , Microfibrilas/genética , Animais , Linhagem Celular , Ectopia do Cristalino/genética , Ectopia do Cristalino/patologia , Células Epiteliais/metabolismo , Células Epiteliais/patologia , Fibrilina-1 , Fibrilinas , Técnicas de Inativação de Genes , Marcação de Genes , Genótipo , Glaucoma/genética , Humanos , Proteínas de Ligação a TGF-beta Latente/metabolismo , Camundongos , Camundongos Knockout , Proteínas dos Microfilamentos/metabolismo , Mutação , Fenótipo , Ligação ProteicaRESUMO
Elastic fiber assembly requires deposition of elastin monomers onto microfibrils, the mechanism of which is incompletely understood. Here we show that latent TGF-ß binding protein 4 (LTBP-4) potentiates formation of elastic fibers through interacting with fibulin-5, a tropoelastin-binding protein necessary for elastogenesis. Decreased expression of LTBP-4 in human dermal fibroblast cells by siRNA treatment abolished the linear deposition of fibulin-5 and tropoelastin on microfibrils. It is notable that the addition of recombinant LTBP-4 to cell culture medium promoted elastin deposition on microfibrils without changing the expression of elastic fiber components. This elastogenic property of LTBP-4 is independent of bound TGF-ß because TGF-ß-free recombinant LTBP-4 was as potent an elastogenic inducer as TGF-ß-bound recombinant LTBP-4. Without LTBP-4, fibulin-5 and tropoelastin deposition was discontinuous and punctate in vitro and in vivo. These data suggest a unique function for LTBP-4 during elastic fibrogenesis, making it a potential therapeutic target for elastic fiber regeneration.
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Proteínas da Matriz Extracelular/metabolismo , Proteínas de Ligação a TGF-beta Latente/fisiologia , Proteínas Recombinantes/metabolismo , Animais , Células HEK293 , Humanos , Proteínas de Ligação a TGF-beta Latente/metabolismo , Camundongos , Camundongos Knockout , Ligação Proteica , Interferência de RNARESUMO
A novel series of 2-pyridyl-benzensulfonamide derivatives have been identified as selective and orally active TRPM8 antagonists via high throughput screening (HTS). Exploration of the structure-activity relationships of compound 1 has led to the identification of RQ-00203078 (compound 36) as a highly selective, potent and orally available TRPM8 antagonist. RQ-00203078 demonstrated excellent in vivo activity in a dose dependent manner with an ED50 value of 0.65 mg/kg in the icilin-induced wet-dog shakes model in rats after oral administration and may become an important pharmacological tool for fully assessing the potential therapeutic use of the targets activated by cold stimulation.
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Canais de Cátion TRPM/antagonistas & inibidores , Animais , Estrutura Molecular , Ratos , Relação Estrutura-Atividade , Canais de Cátion TRPM/genética , Canais de Cátion TRPM/metabolismoRESUMO
We have built equipment that can visualize the angle distributions of scattered X-rays. The main body of the equipment is made of a lead-shielded box 340 mm long, 300 mm wide and 270 mm high. The collimated X-rays are introduced into the equipment from the front face, then scattered by the sample located in the center of the equipment. The X-rays scattering toward the upper side are detected by the phosphor plate. To verify the usability of the equipment, an experiment using diagnostic X-rays was carried out. X-rays with a tube voltage of 100 kV were narrowed down to 6 mm(phi) and a 2 mm-thick acrylic sample was irradiated. The experimental conditions with a tube current-time product of 300 mAs to 1500 mAs proved appropriate for obtaining suitable images on a 10 inch x 12 inch phosphor plate. The obtained images were analyzed using ImageJ. The experimental values were in good agreement with the theoretical distribution calculated by Klein and Nishina. Because the distribution of the scattered X-rays can be visualized in relatively simple experiments using the developed equipment, it is hoped that it will be of use for the practical training of beginners.
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Espalhamento de Radiação , Tecnologia Radiológica/educação , Materiais de EnsinoRESUMO
The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease's rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease.