MONA

蒙娜
  • 文章类型: Journal Article
    钠掺杂的钼(MoNa)在形成MoSe2界面层的关键影响,影响CIGSe/Mo异质结构的电性能,并达到最佳的MoSe2形成条件,提高了异质接触质量。值得注意的是,具有600nm厚的MoNa层的样品显示出最高的电阻率(73μΩcm)和薄层电阻(0.45Ω/平方),强调了MoNa层厚度对电导率的实质性影响。通过MoNa层的受控钠扩散对于实现所需的电特性至关重要,影响Na扩散速率,晶粒尺寸,和整体形态,正如EDX和FESEM分析所阐明的那样。此外,XRD结果提供了对自发剥离现象的见解,样品具有〜600nm的MoNa层,显示出最强的衍射峰和最大的晶体尺寸,表明钠的存在促进了Mo向MoSe2的转化增强。拉曼光谱进一步证实了MoSe2的存在,其厚度与MoNa层厚度相关。随着MoSe2层厚度的增加,观察到的电阻增加和电导率降低,突显了最佳MoSe2形成对于CIGSe/Mo异质结构中从肖特基过渡到欧姆接触的至关重要性。最终,讨论了促进CIGSe薄膜太阳能电池生产的重要因素,提供对MoNa和MoSe2相互作用的细致入微的见解,阐明它们对CIGSe/Mo异质结构的电特性的集体影响。
    The critical impact of sodium-doped molybdenum (MoNa) in shaping the MoSe2 interfacial layer, influencing the electrical properties of CIGSe/Mo heterostructures, and achieving optimal MoSe2 formation conditions, leading to improved hetero-contact quality. Notably, samples with a 600-nm-thick MoNa layer demonstrate the highest resistivity (73 μΩcm) and sheet resistance (0.45 Ω/square), highlighting the substantial impact of MoNa layer thickness on electrical conductivity. Controlled sodium diffusion through MoNa layers is essential for achieving desirable electrical characteristics, influencing Na diffusion rates, grain sizes, and overall morphology, as elucidated by EDX and FESEM analyses. Additionally, XRD results provide insights into the spontaneous peeling-off phenomenon, with the sample featuring a ~ 600-nm MoNa layer displaying the strongest diffraction peak and the largest crystal size, indicative of enhanced Mo to MoSe2 conversion facilitated by sodium presence. Raman spectra further confirm the presence of MoSe2, with its thickness correlating with MoNa layer thickness. The observed increase in resistance and decrease in conductivity with rising MoSe2 layer thickness underscore the critical importance of optimal MoSe2 formation for transitioning from Schottky to ohmic contact in CIGSe/Mo heterostructures. Ultimately, significant factors to the advancement of CIGSe thin-film solar cell production are discussed, providing nuanced insights into the interplay of MoNa and MoSe2, elucidating their collective impact on the electrical characteristics of CIGSe/Mo heterostructures.
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  • 文章类型: Journal Article
    在美国和世界范围内,甾醇去甲基化抑制剂(DMI)杀真菌剂仍然是控制由果莫尼氏菌引起的桃棕腐烂的必要成分。在美国东南部,对DMIs的抗性与细胞色素P45014α-脱甲基酶基因MfCYP51以及遗传元件Mona的过表达有关,在抗性分离株中位于MfCYP51上游的长度为65bp的核苷酸序列。第一次调查大约20年后,我们重新评估了来自南卡罗来纳州和乔治亚州的果粒分枝杆菌对丙环唑的敏感性,并首次评估了来自阿拉巴马州的分离株。从各种商业果园和两个实验果园共收集了238株果粒分离株,并根据0.3µg/ml的区分剂量确定了对丙环唑的敏感性。结果显示16.2%,89.2%和72.4%的分离株来自阿拉巴马州,格鲁吉亚,和南卡罗来纳州,分别,对丙环唑耐药。阿拉巴马州的耐药性检测是该州的第一份报告。所有耐药分离株都含有莫娜,但大多数敏感分离株都没有。目前尚不清楚南卡罗来纳州和乔治亚州的抵抗频率是否增加。然而,抗性水平(通过基于剂量的相对生长类别中的分离频率评估)没有显着变化,没有发现其他抗性基因型的证据。对抗性分离株CF010中上游MfCYP51基因区域的分析揭示了本报告中首次描述的插入序列。我们的研究表明,当前的杀真菌剂喷雾计划已有效地防止了果蝇种群中抗性水平的增加,并抑制了病原体的新抗性基因型的发展。
    Sterol demethylation inhibitor (DMI) fungicides continue to be essential components for the control of brown rot of peach caused by Monilinia fructicola in the United States and worldwide. In the southeastern United States, resistance to DMIs had been associated with overexpression of the cytochrome P450 14α-demethylase gene MfCYP51 as well as the genetic element Mona, a 65 bp in length nucleotide sequence located upstream of MfCYP51 in resistant isolates. About 20 years after the first survey, we reevaluated sensitivity of M. fructicola from South Carolina and Georgia to propiconazole and also evaluated isolates from Alabama for the first time. A total of 238 M. fructicola isolates were collected from various commercial and two experimental orchards, and sensitivity to propiconazole was determined based on a discriminatory dose of 0.3 μg/ml. Results indicated 16.2, 89.2, and 72.4% of isolates from Alabama, Georgia, and South Carolina, respectively, were resistant to propiconazole. The detection of resistance in Alabama is the first report for the state. All resistant isolates contained Mona, but it was absent from most sensitive isolates. It was unclear if the resistance frequency had increased in South Carolina and Georgia. However, the resistance levels (as assessed by the isolate frequency in discriminatory dose-based relative growth categories) did not change notably, and no evidence of other resistance genotypes was found. Analysis of the upstream MfCYP51 gene region in the resistant isolate CF010 revealed an insertion sequence described for the first time in this report. Our study suggests that current fungicide spray programs have been effective against increasing resistance levels in populations of M. fructicola and suppressing development of new resistant genotypes of the pathogen.
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  • 文章类型: Case Reports
    Frank-TerHaar综合征(FTHS),温彻斯特综合征(WS),Torg综合征(TS)和多中心性骨质溶解结节病和关节病(MONA)是进行性骨骼发育不良,包括肢端骨溶解。基质金属蛋白酶2(MMP2)的突变,基质金属蛋白酶14(MMP14)和SH3PXD2B是这些疾病中已知的遗传缺陷。我们特此报告一名患有进行性肢体畸形的5岁零9个月大女孩。她是一对亲戚的第一个孩子,由于生长不良和骨痛,他被转诊到代谢紊乱诊所。在体检时,轻微的面部畸形,多毛症,严重的手部畸形,腕关节活动范围有限,掌骨和指骨关节,足外翻畸形,检测到掌足底区域的软组织肥大和结节形成。她过去的病史表明心脏缺陷导致8个月大的心脏直视手术。遗传研究揭示了MMP2基因中一个新的纯合子无义突变,解释了她的临床表现。我们建议对先天性心脏病患者进行仔细的评估和随访,因为这可能是遗传性多系统疾病的首次出现。早期将疾病与其他骨骼发育不良和风湿病区分开来可以防止不必要的治疗。
    Frank-Ter Haar syndrome (FTHS), Winchester syndrome (WS), Torg syndrome (TS) and Multicentric Osteolysis Nodulosis and Arthropathy (MONA) are progressive skeletal dysplasia consisting of acro-osteolysis. Mutation in Matrix Metalloproteinase 2 (MMP2), Matrix Metalloproteinase 14 (MMP14) and SH3PXD2B are known genetic defects in these disorders. We hereby report a 5 years and 9 months old girl suffering from progressive limb deformity. She is the first child of a relative couple, who was referred to metabolic disorders\' clinic due to poor growth and bone pain. On physical examination, minor facial dysmorphism, hypertrichosis, severe hand deformity with limitation in range of motion in carpal, metacarpal and phalangeal joints, hallux valgus deformity of feet, soft tissue hypertrophy and nodule formation in palmoplantar areas were detected. Her past history indicated a cardiac defect resulting in open heart surgery at 8 months of age. Genetic study revealed a new homozygote nonsense mutation in MMP2 gene explaining her clinical manifestations. We recommend careful evaluation and follow-up of patients with congenital heart disease, as it may be the first presentation of a genetic multisystem disorder. Early differentiation of the disease from other skeletal dysplasia and rheumatologic disorders could prevent unnecessary management.
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  • 文章类型: Case Reports
    Multicentric Osteolysis Nodulosis and Arthropathy (MONA) is an ultra-rare multisystem autosomal recessive disorder characterized by progressive osteolysis, subcutaneous nodules and developing arthropathy. The characteristic radiological signs combined with symptoms resembling juvenile idiopathic arthritis (JIA) set the diagnosis, which is established either by measuring matrix metalloproteinase-2 (MMP-2) enzyme activity through electrophoresis (zymography) or genomic testing. We report the clinical and radiographic findings of a 14-year-old girl with molecularly proven MONA, who presented with painless osteolytic changes of the feet and upper extremities and developed hip arthritis. To this day, no specific therapy has been identified with proven long term relief and control of the disease progression.
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  • 文章类型: Journal Article
    Resistance to sterol demethylation inhibitor (DMI) fungicides in Monilinia fructicola, causal agent of brown rot of stone fruit, has been reported in the southeastern and eastern United States and in Brazil. DMI resistance of some M. fructicola isolates, in particular those recovered from the southeastern United States, is associated with a sequence element termed \"Mona\" that causes overexpression of the cytochrome demethylase target gene MfCYP51. In this study, we conducted statewide surveys of Michigan stone fruit orchards from 2009 to 2011 and in 2019, and we determined the sensitivity to propiconazole of a total of 813 isolates of M. fructicola. A total of 80.7% of Michigan isolates were characterized as resistant to propiconazole by relative growth assays, but the Mona insert was not uniformly detected and was present in some isolates that were not characterized as DMI resistant. Gene expression assays indicated that elevated expression of MfCYP51 was only weakly correlated with DMI resistance in M. fructicola isolates from Michigan, and there was no obvious correlation between the presence of the Mona element and elevated expression of MfCYP51. However, sequence analysis of MfCYP51 from 25 DMI-resistant isolates did not reveal any point mutations that could be correlated with resistance. Amplification and sequencing upstream of MfCYP51 resulted in detection of DNA insertions in a wide range of isolates typed by DMI phenotype and the presence of Mona or other unique sequences. The function of these unique sequences or their presence upstream of MfCYP51 cannot be correlated to a DMI-resistant genotype at this time. Our results indicate that DMI resistance was established in Michigan populations of M. fructicola by 2009 to 2011, and that relative resistance levels have continued to increase to the point that practical resistance is present in most orchards. In addition, the presence of the Mona insert is not a marker for identifying DMI-resistant isolates of M. fructicola in Michigan.
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  • 文章类型: Case Reports
    MONA,代表多心骨质溶解的光谱,皮下结节,和关节病,是一种由基质金属肽酶2(MMP2)基因突变引起的超罕见常染色体隐性遗传疾病。迄今为止只有44个人,已报道携带22种不同的突变。在这里,我们报道了两个具有相同纯合MMP2基因突变的兄弟,但是表型明显不同。
    从受影响的兄弟和父母中分离基因组DNA。从较年轻的患者(索引病例)中进行了骨活检。使用明胶酶谱分析了年轻患者血清和滑液中基质金属肽酶2(MMP2)的水平。
    DNA分析揭示了基因外显子8上的纯合c.118.8C>A颠倒。受影响的兄弟具有相同的纯合变体,并且父母对该变体是杂合的。该变体已被报道为一个个体上的复合杂合突变,导致硬皮病样皮肤增厚。骨组织形态计量学提示骨小梁重塑和更新增加。酶谱显示,血清中MMP2的水平完全无法测量,并且在滑液中仅发现了分子量与MMP2相似的少量明胶溶解蛋白带。
    尽管基因型相同,但这两个兄弟的发病年龄和综合征的表型严重程度都不同。年轻患者的角膜混浊导致视力下降。第一次在这种疾病中,混浊通过角膜移植成功治疗。
    MONA, which stands for a spectrum of Multicentric Osteolysis, subcutaneous Nodulosis, and Athropathia, is an ultra rare autosomal recessive disorder caused by mutations in the matrix metallopeptidase 2 (MMP2) gene. To date only 44 individuals, carrying 22 different mutations have been reported. Here we report on two brothers with identical homozygous MMP2 gene mutations, but with clearly different phenotypes.
    Genomic DNA was isolated from the affected brothers and the parents. An iliac crest bone biopsy was taken from the younger patient (index case). The level of matrix metallopeptidase 2 enzyme (MMP2) in serum and synovial fluid of the younger patient was analyzed using gelatin zymography.
    The DNA analysis revealed a homozygous c.1188C>A transversion on exon 8 of the gene. The affected brothers had the same homozygous variant and the parents were heterozygous to this variant. This variant has been reported as a compound heterozygous mutation on one individual resulting in scleroderma like skin thickening. Bone histomorphometry indicated increased trabecular bone remodeling and turnover. The zymography revealed that the level of MMP2 was completely nonmeasurable in the serum and only a minor gelatinolytic protein band of about similar molecular weight as MMP2 was found in the synovial fluid.
    Both the age at the onset and the phenotypic severity of the syndrome in these two brothers were different despite identical genotypes. The younger patients had corneal opacities leading to deteriorating visual acuity. For the first time in this disease, opacities were successfully treated with corneal transplantations.
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  • 文章类型: Journal Article
    Little is known about the natural history of the Sphaerodactylus species endemic to the three islands located in the Mona Passage separating the Greater Antillean islands of Hispaniola and Puerto Rico. In this study, parts of two mitochondrial genes, 16S rRNA and 12S rRNA, were sequenced to determine the relationships between the sphaerodactylids that live in the Mona Passage and other Caribbean species from the same genus. While the main goal was to identify the biogeographical origin of these species, we also identified a genetically distinct type of dwarf gecko that warrants future evaluation as a possible new species. According to the reconstructed phylogenies, we propose a stepwise model of colonization wherein S. nicholsi from southwestern Puerto Rico or a very close ancestor gave rise through a founder event to Sphaerodactylus monensis on Mona Island. In a similar fashion, S. monensis or a very close ancestor on Mona Island gave rise to S. levinsi on Desecheo Island. This study also suggests that the most recent common ancestor between the species from the islands in the Mona Passage and Puerto Rico existed approximately 3 MYA.
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