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51.
Arisa Ueki Kokichi Sugano Kumiko Misu Eriko Aimono Kohei Nakamura Shigeki Tanishima Nobuyuki Tanaka Shuji Mikami Akira Hirasawa Miho Ando Teruhiko Yoshida Mototsugu Oya Hiroshi Nishihara Kenjiro Kosaki 《International journal of molecular sciences》2021,22(15)
Hereditary leiomyomatosis and renal cell carcinoma (HL (RCC)) entails cutaneous and uterine leiomyomatosis with aggressive type 2 papillary RCC-like histology. HLRCC is caused by pathogenic variants in the FH gene, which encodes fumarate hydratase (FH). Here, we describe an episode of young-onset RCC caused by a genomic FH deletion that was diagnosed via clinical sequencing. A 35-year-old woman was diagnosed with RCC and multiple metastases: histopathological analyses supported a diagnosis of FH-deficient RCC. Although the patient had neither skin tumors nor a family history of HLRCC, an aggressive clinical course at her age and pathological diagnosis of FH-deficient RCC suggested a germline FH variant. After counseling, the patient provided written informed consent for germline genetic testing. She was simultaneously subjected to paired tumor profiling tests targeting the exome to identify a therapeutic target. Although conventional germline sequencing did not detect FH variants, exome sequencing revealed a heterozygous germline FH deletion. As such, paired tumor profiling, not conventional sequencing, was required to identify this genetic deletion. RCC caused by a germline FH deletion has hitherto not been described in Japan, and the FH deletion detected in this patient was presumed to be of maternal European origin. Although the genotype-phenotype correlation in HLRCC-related tumors is unclear, the patient’s family was advised to undergo genetic counseling to consider additional RCC screening. 相似文献
52.
基于优化自组织聚类神经网络的入侵检测方法研究 总被引:1,自引:0,他引:1
提出一种将自组织聚类神经网络运用于入侵检测的方法。在这种方法中采用自适应谐振学习算法进行训练;当网络的平均误差不再有意义地减少时,用遗传算法对网络继续进行训练得到最佳权值。用神经网络和遗传算法使网络结构和网络连接权值同时进化,收敛性好,自适应性强,适合于实时处理。仿真结果表明该网络取得良好检测效果。 相似文献
53.
Growth hormone (GH) plays an important role in auditory development during the embryonic stage. Exogenous agents such as sound, noise, drugs or trauma, can induce the release of this hormone to perform a protective function and stimulate other mediators that protect the auditory pathway. In addition, GH deficiency conditions hearing loss or central auditory processing disorders. There are promising animal studies that reflect a possible regenerative role when exogenous GH is used in hearing impairments, demonstrated in in vivo and in vitro studies, and also, even a few studies show beneficial effects in humans presented and substantiated in the main text, although they should not exaggerate the main conclusions. 相似文献
54.
55.
Roberta Zuntini Elena Bonora Laura Maria Pradella Laura Benedetta Amato Michele Vidone Sara De Fanti Irene Catucci Laura Cortesi Veronica Medici Simona Ferrari Giuseppe Gasparre Paolo Peterlongo Marco Sazzini Daniela Turchetti 《International journal of molecular sciences》2021,22(11)
The NBN gene has been included in breast cancer (BC) multigene panels based on early studies suggesting an increased BC risk for carriers, though not confirmed by recent research. To evaluate the impact of NBN analysis, we assessed the results of NBN sequencing in 116 BRCA-negative BC patients and reviewed the literature. Three patients (2.6%) carried potentially relevant variants: two, apparently unrelated, carried the frameshift variant c.156_157delTT and another one the c.628G>T variant. The latter was subsequently found in 4/1390 (0.3%) BC cases and 8/1580 (0.5%) controls in an independent sample, which, together with in silico predictions, provided evidence against its pathogenicity. Conversely, the rare c.156_157delTT variant was absent in the case-control set; moreover, a 50% reduction of NBN expression was demonstrated in one carrier. However, in one family it failed to co-segregate with BC, while the other carrier was found to harbor also a probably pathogenic TP53 variant that may explain her phenotype. Therefore, the c.156_157delTT, although functionally deleterious, was not supported as a cancer-predisposing defect. Pathogenic/likely pathogenic NBN variants were detected by multigene panels in 31/12314 (0.25%) patients included in 15 studies. The risk of misinterpretation of such findings is substantial and supports the exclusion of NBN from multigene panels. 相似文献
56.
Alejandra Gonzalez-Duarte Alfredo Ulloa-Aguirre 《International journal of molecular sciences》2021,22(23)
Transthyretin (TTR) amyloidogenesis involves the formation, aggregation, and deposition of amyloid fibrils from tetrameric TTR in different organs and tissues. While the result of amyloidoses is the accumulation of amyloid fibrils resulting in end-organ damage, the nature, and sequence of the molecular causes leading to amyloidosis may differ between the different variants. In addition, fibril accumulation and toxicity vary between different mutations. Structural changes in amyloidogenic TTR have been difficult to identify through X-ray crystallography; but nuclear magnetic resonance spectroscopy has revealed different chemical shifts in the backbone structure of mutated and wild-type TTR, resulting in diverse responses to the cellular conditions or proteolytic stress. Toxic mechanisms of TTR amyloidosis have different effects on different tissues. Therapeutic approaches have evolved from orthotopic liver transplants to novel disease-modifying therapies that stabilize TTR tetramers and gene-silencing agents like small interfering RNA and antisense oligonucleotide therapies. The underlying molecular mechanisms of the different TTR variants could be responsible for the tropisms to specific organs, the age at onset, treatment responses, or disparities in the prognosis. 相似文献
57.
Ikenaka K Katsuno M Kawai K Ishigaki S Tanaka F Sobue G 《International journal of molecular sciences》2012,13(1):1225-1238
Motor neurons typically have very long axons, and fine-tuning axonal transport is crucial for their survival. The obstruction of axonal transport is gaining attention as a cause of neuronal dysfunction in a variety of neurodegenerative motor neuron diseases. Depletions in dynein and dynactin-1, motor molecules regulating axonal trafficking, disrupt axonal transport in flies, and mutations in their genes cause motor neuron degeneration in humans and rodents. Axonal transport defects are among the early molecular events leading to neurodegeneration in mouse models of amyotrophic lateral sclerosis (ALS). Gene expression profiles indicate that dynactin-1 mRNA is downregulated in degenerating spinal motor neurons of autopsied patients with sporadic ALS. Dynactin-1 mRNA is also reduced in the affected neurons of a mouse model of spinal and bulbar muscular atrophy, a motor neuron disease caused by triplet CAG repeat expansion in the gene encoding the androgen receptor. Pathogenic androgen receptor proteins also inhibit kinesin-1 microtubule-binding activity and disrupt anterograde axonal transport by activating c-Jun N-terminal kinase. Disruption of axonal transport also underlies the pathogenesis of spinal muscular atrophy and hereditary spastic paraplegias. These observations suggest that the impairment of axonal transport is a key event in the pathological processes of motor neuron degeneration and an important target of therapy development for motor neuron diseases. 相似文献
58.
Francisco J. Padilla-Godínez Rodrigo Ramos-Acevedo Hilda Anglica Martínez-Becerril Luis D. Bernal-Conde Jernimo F. Garrido-Figueroa Marcia Hiriart Adriana Hernndez-Lpez Rubn Argüero-Snchez Francesco Callea Magdalena Guerra-Crespo 《International journal of molecular sciences》2021,22(22)
Dysfunction of cellular homeostasis can lead to misfolding of proteins thus acquiring conformations prone to polymerization into pathological aggregates. This process is associated with several disorders, including neurodegenerative diseases, such as Parkinson’s disease (PD), and endoplasmic reticulum storage disorders (ERSDs), like alpha-1-antitrypsin deficiency (AATD) and hereditary hypofibrinogenemia with hepatic storage (HHHS). Given the shared pathophysiological mechanisms involved in such conditions, it is necessary to deepen our understanding of the basic principles of misfolding and aggregation akin to these diseases which, although heterogeneous in symptomatology, present similarities that could lead to potential mutual treatments. Here, we review: (i) the pathological bases leading to misfolding and aggregation of proteins involved in PD, AATD, and HHHS: alpha-synuclein, alpha-1-antitrypsin, and fibrinogen, respectively, (ii) the evidence linking each protein aggregation to the stress mechanisms occurring in the endoplasmic reticulum (ER) of each pathology, (iii) a comparison of the mechanisms related to dysfunction of proteostasis and regulation of homeostasis between the diseases (such as the unfolded protein response and/or autophagy), (iv) and clinical perspectives regarding possible common treatments focused on improving the defensive responses to protein aggregation for diseases as different as PD, and ERSDs. 相似文献
59.
The magnesium ingots from factory A, B and C were remelted. The microstructure and property of un-remelted and remelted magnesium ingots were investigated. The results indicate that the microstructure and property of the remelted magnesium alloy take on a hereditary transmission similar or resembling to those of feed materials. The characteristics of the short range order structure atomic group in alloy is the same as or resembles to that of feed materials during the melting process. The hereditary transmission of magnesium alloy can be explained by atomic conglobation theory. 相似文献
60.
用改进的遗传算法进行地震波阻抗反演研究 总被引:18,自引:1,他引:18
本文介绍的遗传算法是在自然界生物遗传过程优选和进化的基础上,针对地震波阻抗反演的实际问题,对传统的遗传算法进行改进,取消传统的二进制编程,解码过程,直接使用十进制实数进行运算,在对反演参数的约束方面,采用约束法,不仅大大简化了算法的过程,而且使收敛速度大大加快,使用改进的算法对地震波阻抗进行反演,可使目标函数达到10^-4数量级,这就使遗传算法的非线性化方法可应用于地震波阻抗反演中。 相似文献