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1.
Sci Rep ; 14(1): 17718, 2024 Jul 31.
Artículo en Inglés | MEDLINE | ID: mdl-39085252

RESUMEN

Risks in the supply chain can damage many companies and organizations due to sustainability risk factors. This study evaluates the supply chain risk assessment and management and then selects the best supplier in a gas company in Egypt. A comprehensive methodology can use the experts' opinions who use the linguistic variables in the spherical fuzzy numbers (SFNs) to evaluate the criteria and suppliers in this study based on their views. Selecting the best supplier is a complex task due to various criteria related to supply chain risk assessment, such as supply risks, environmental risks, financial risks, regularity risks, political risk, ethical risks, and technology risks and their sub-criteria. This study suggested a new combined model with multi-criteria decision-making (MCDM) under a spherical fuzzy set (SFS) environment to overcome uncertainty and incomplete data in the assessment process. The MCDM methodology has two methods: the Entropy and COmbinative Distance-based Assessment (CODAS) methods. The SFS-Entropy is used to compute supply chain risk assessment and management criteria weights. The SFS-CODAS method is used to rank the supplier. The main results show that supply risks have the highest importance, followed by financial and environmental risks, and ethical risks have the lowest risk importance. The criteria weights were changed under sensitivity analysis to show the stability and validation of the results obtained from the suggested methodology. The comparative analysis is implemented with other MCDM methods named TOPSIS, VIKOR, MARCOS, COPRAS, WASPAS, and MULTIMOORA methods under the SFS environment. This study can help managers and organizations select the best supplier with the lowest sustainability risks.

2.
J Environ Manage ; 345: 118838, 2023 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-37595460

RESUMEN

Flood risk assessment is a key step in flood management and mitigation, and flood risk maps provide a quantitative measure of flood risk. Therefore, integration of deep learning - an updated version of machine learning techniques - and multi-criteria decision making (MCDM) models can generate high-resolution flood risk maps. In this study, a novel integrated approach has been developed based on multiplicative long short-term memory (mLSTM) deep learning models and an MCDM ensemble model to map flood risk in the Minab-Shamil plain, southern Iran. A flood hazard map generated by the mLSTM model is based on nine critical features selected by GrootCV (distance to the river, vegetation cover, variables extracted from DEM (digital elevation model) and river density) and a flood inventory map (70% and 30% data were randomly selected as training and test datasets, respectively). The values of all criteria used to assess model accuracy performance (except Cohens kappa for train dataset = 86, and for test dataset = 84) achieved values greater than 90, which indicates that the mLSTM model performed very well for the generation of a spatial flood hazard map. According to the spatial flood hazard map produced by mLSTM, the very low, low, moderate, high and very high classes cover 26%, 35.3%, 20.5%, 11.2% and 7% of the total area, respectively. Flood vulnerability maps were produced by the combinative distance-based assessment (CODAS), the evaluation based on distance from average solution (EDAS), and the multi-objective optimization on the basis of simple ratio analysis (MOOSRA), and then validated by Spearman's rank correlation coefficients (SRC). Based on the SRC, the three models CODAS, EDAS, and MOOSRA showed high-ranking correlations with each other, and all three models were then used in the ensemble process. According to the CODAS-EDAS-MOOSRA ensemble model, 21.5%, 34.2%, 23.7%, 13%, and 7.6% of the total area were classified as having a very low to very high flood vulnerability, respectively. Finally, a flood risk map was generated by the combination of flood hazard and vulnerability maps produced by the mLSTM and MCDM ensemble model. According to the flood risk map, 27.4%, 34.3%, 14.8%, 15.7%, and 7.8% of the total area were classified as having a very low, low, moderate, high, and very high flood risk, respectively. Overall, the integration of mLSTM and the MCDM ensemble is a promising tool for generating precise flood risk maps and provides a useful reference for flood risk management.


Asunto(s)
Aprendizaje Profundo , Inundaciones , Memoria a Corto Plazo , Medición de Riesgo , Toma de Decisiones
3.
Radiol Case Rep ; 18(3): 1000-1004, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36684615

RESUMEN

CODAS syndrome (cerebral, ocular, dental, auricular, skeletal anomalies) is a rare autosomal recessive inherited multisystemic disease that carries an incidence rate of less than 1 in 1,000,000 children worldwide. It has an infancy, neonatal age of onset, characterized by deformities of the central nervous system, eyes, ears, teeth, and skeleton. A 1-year-old female of non-consanguineous parents, first time presented to our pediatrics clinic on November 6, 2021 when she was 4 months of age with developmental delay, as the patient could not support her head and made no eye contact on examination. Microcephaly was observed. She had a positive family history; her sister died at the age of 3 days with microcephaly and diaphragmatic hernia. We recommend that a wider range of centers to get encouraged to report cases of CODAS they might encounter due to the lack of sufficient amounts if solid literature on the topic. To our knowledge, this is the first case to be reported in the literature from Saudi Arabia.

4.
Soft comput ; 27(5): 2325-2345, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36570599

RESUMEN

The selection of a proper international freight transport route is one of the crucial tasks for decision-makers since it can affect costs, efficiency, and transportation performance. Besides, the selection of suitable and appropriate freight routes can also reduce external costs of transportation such as emissions, noise, traffic congestions, accidents, and so on. Route selection in international transportation is a complicated decision-making problem as many conflicting factors and criteria affect the assessment process. It has been observed that there is no mathematical model and methodological frame used for solving these selection problems, and decision-makers make decisions on this issue based on their own experiences and verbal judgments in the research process. Therefore, a methodological frame is required to make rational, realistic, and optimal decisions on route selection. From this perspective, the current paper proposes using the IVAIF CODAS, an extended version of the traditional CODAS techniques, and using the Atanassov interval-valued intuitionistic fuzzy sets (IVAIFS) for processing better the existing uncertainties. The proposed model is applied to solve the route selection, a real-life decision-making problem encountered in international transportation between EU countries and Turkey. According to the results of the analysis, option A6 (i.e., Route-6 (Bursa-Istanbul-Pendik-Trieste (Ro-Ro)-Austria-Frankfurt/Germany) has been determined as the best alternative. These obtained results have been approved by a comprehensive sensitivity analysis performed by using different MCDM techniques based on interval-valued intuitionistic fuzzy sets. Hence, it can be accepted that the proposed model is an applicable, robust, and powerful mathematical tool; also, it can provide very reliable, accurate, and reasonable results. As a result, the proposed model can provide a more flexible and effective decision-making environment as well as it can provide valuable advantages to the logistics and transport companies for carrying out practical, productive, and lower cost logistics operations.

5.
Clin Linguist Phon ; 37(9): 802-827, 2023 09 02.
Artículo en Inglés | MEDLINE | ID: mdl-35876441

RESUMEN

In this paper, we present a case study of an atypically developing Hebrew-acquiring boy (YV), in comparison with three typically developing boys. Drawing on data from longitudinal studies, we examined the development of two verbal suffixes, -im 'ms.pl.pres' and -ti '1.sg.past', with reference to two prosodic structures that these suffixes assume - word final codas for -im and trisyllabic words for -ti (e.g. bon-ím 'they build', kará-ti 'I read'). We found that YV's developmental trajectory was similar to that of the three boys in both phonology and morphology, each module independently; the deviant phenomena were found at the interface between phonology and morphology. The typically developing boys produced the relevant phonological structures in bare stems before they produced them in suffixed forms. YV, however, proceeded in the opposite order; he produced the final m in -im verbs before mastering word final codas in bare stems; similarly, he produced trisyllabic forms in -ti verbs before mastering them in bare stems. We attribute this deviance to asynchronization between phonological (prosodic) and morphological development. That is, YV's phonology lagged behind his morphology, but this lag did not block the morphological development as it would have in synchronized development.


Asunto(s)
Enfermedades Hereditarias del Ojo , Disco Óptico , Masculino , Humanos , Lingüística , Presión
6.
Int J Mol Sci ; 23(21)2022 Nov 02.
Artículo en Inglés | MEDLINE | ID: mdl-36362158

RESUMEN

Recent studies have indicated a central role for LonP1 in mitochondrial function. Its physiological functions include proteolysis, acting as a molecular chaperone, binding mitochondrial DNA, and being involved in cellular respiration, cellular metabolism, and oxidative stress. Given its vital role in energy metabolism, LonP1 has been suggested to be associated with multi-system neoplasms and developmental disorders. In this study, we investigated the roles, possible mechanisms of action, and therapeutic roles of LonP1 in oral and maxillofacial tumor development. LonP1 was highly expressed in oral-maxillofacial cancers and regulated their development through a sig-naling network. LonP1 may therefore be a promising anticancer therapy target. Mutations in LONP1 have been found to be involved in the etiology of cerebral, ocular, dental, auricular, and skeletal syndrome (CODAS). Only patients carrying specific LONP1 mutations have certain dental abnormalities (delayed eruption and abnormal morphology). LonP1 is therefore a novel factor in the development of oral and maxillofacial tumors. Greater research should therefore be conducted on the diagnosis and therapy of LonP1-related diseases to further define LonP1-associated oral phenotypes and their underlying molecular mechanisms.


Asunto(s)
Proteínas Mitocondriales , Neoplasias , Humanos , Proteasas ATP-Dependientes/metabolismo , Proteínas Mitocondriales/genética , Proteínas Mitocondriales/metabolismo , Mitocondrias/metabolismo , ADN Mitocondrial/metabolismo
7.
Chemosphere ; 308(Pt 3): 136328, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36130647

RESUMEN

The transition towards a sustainable land management (SLM) needs for utilizing appropriate technologies for controlling soil pollution, and ensures the development of environmental, economic, technical, and social dimensions in region. Using these technologies for cleaning up soils polluted with oil and petroleum products in Behbahan city (in the southwest of Iran) is considered. The soil pollution control technologies (SPCTs) can assist local authorities, governments, investors, and developers to reduce climate change, mitigate soil, water, and air pollution and construct the sustainable communities. With the aim of balance between the issues in the context of sustainability policy, criteria (principles) including environmental, economic, technical, and social aspects are considered. The research takes into consideration the applicability of a novel multi-criteria decision-making approach namely complex spherical fuzzy set-based CODAS (Combinative distance-based assessment). The approach can be used to choose the suitable technology in a sustainable mode, considering the principles related to sustainability pillars. It comprises various technologies for soil pollution control and prioritizes technologies from the most to least as: Biorestoration technology, Excavation technology, Soil venting technology, Soil flushing, and Hydraulic barriers technology.


Asunto(s)
Contaminación por Petróleo , Petróleo , Contaminantes del Suelo , Contaminación por Petróleo/prevención & control , Suelo , Contaminantes del Suelo/análisis , Tecnología , Contaminación del Agua
8.
Am J Med Genet A ; 188(9): 2819-2824, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35779070

RESUMEN

EVEN-PLUS syndrome is a rare autosomal recessive disorder caused by biallelic pathogenic variants in the mitochondrial chaperone called mortalin, encoded by HSPA9. This genetic disorder, presenting with several overlapping features with CODAS syndrome, is characterized by the involvement of the Epiphyses, Vertebrae, Ears, and Nose (EVEN), PLUS associated findings. Only five individuals presenting with the EVEN-PLUS phenotype and biallelic variants in HSPA9 have been published. Here, we expand the phenotypic and molecular spectrum associated with this disorder, reporting two sibs with a milder phenotype and compound heterozygous pathogenic variants (a recurrent variant and a novel one). Also, we confirm a homozygous pathogenic variant in the family originally reported as EVE dysplasia.


Asunto(s)
Anomalías Craneofaciales , Osteocondrodisplasias , Anomalías Dentarias , Anomalías Craneofaciales/diagnóstico , Anomalías Craneofaciales/genética , Proteínas HSP70 de Choque Térmico/genética , Homocigoto , Humanos , Proteínas Mitocondriales/genética , Osteocondrodisplasias/diagnóstico , Osteocondrodisplasias/genética , Fenotipo
9.
Materials (Basel) ; 15(12)2022 Jun 08.
Artículo en Inglés | MEDLINE | ID: mdl-35744144

RESUMEN

End-milling operation of steel grade material is a challenging task as it is hard-to-cut material. Proper selection of cutting tools, cutting conditions, and cutting process parameters is important to improve productivity, surface quality, and tool life. Therefore, the present study investigated the end-milling operation of AISI 1522H steel grade under minimum-quantity lubrication (MQL) conditions using a novel blend of vegetable oils, namely canola and olive oil. Cutting process parameters considered were spindle speed (s), feed rate (f), depth of cut (d), width of cut (w), and cutting conditions (c), while responses were average surface roughness (Ra), cutting forces (Fc), tool wear (TW), and material removal rate (MRR). Experimental runs were designed based on the definitive screening design (DSD) method. Analysis of variance (ANOVA) results show that feed rate significantly affects all considered responses. Nonlinear prediction models were developed for each response variable, and their validity was also verified. Finally, multi-response optimization was performed using the combinative distance-based assessment (CODAS) method coupled with criteria importance through inter-criteria correlation (CRITIC). The optimized parameters found were: s = 1200 rpm, f = 320 mm/min, d = 0.6 mm, w = 8 mm, and c = 100 mL/h. Further, it was compared with other existing multi-response optimization methods and induced good results.

10.
Waste Manag ; 143: 1-14, 2022 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-35217384

RESUMEN

Recently, the novel concept of the circular supply chain (CSC) has gained substantial attention amongst researchers across the globe. It is due to the issues of unsustainability in supply chain operation of manufacturing industry. CSC amalgamates the circular economy (CE) into the supply chain of manufacturing organizations. Business organizations can achieve several sustainable development goals by adopting CSC as an innovative strategy. However, limited attention has been given to its implementation in emerging economies. Thus, this research aim is to identify and analyze the essential CE practices that help to accomplish the several sustainable development goals (SDGs) of CSC management. This research presents an integrated framework of Pythagorean fuzzy analytic hierarchy process (PF-AHP) and Pythagorean fuzzy combinative distance-based assessment (PF-CODAS) techniques. PF-AHP is employed to determine the relative importance of CE practices, whereas PF-CODAS method ranks the SDGs derived due to the adoption of CE practices. The effectiveness of the proposed framework is validated with the help of an Indian manufacturing organization. The finding of this research reveals that practices based on 'government', 'management', and 'economy' initiatives play a significant role and contribute 50 % of its influence on the effective CSC adoption, whereas, 'mitigate waste and enhance environmental sustainability', is identified as the most critical SDG realized due to adoption of CE practices. Sensitivity analysis is conducted to check the robustness of proposed methods. This research provides the systematic, accurate, and valuable decision support tools to practitioners to execute the CE practices efficiently for achieving the various sustainability goals.


Asunto(s)
Comercio , Desarrollo Sostenible
11.
Front Genet ; 13: 1031856, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36685982

RESUMEN

Background: CODAS syndrome (MIM 600373) is a multi-system developmental disorder characterized by cerebral, ocular, dental, auricular, and skeletal anomalies. CODAS syndrome is rare in the world and no cases have been reported in Chinese population so far. Mutations in the LONP1 gene can contribute to CODAS syndrome, while the underlying molecular mechanisms requires further investigation. Method: We described a Chinese boy who has suffered from cognition impairment, cataracts, caries, abnormal auricle and skeletal anomalies since birth. The patient's parents are non-consanguineous and healthy. Whole-exome sequencing (WES) was employed to explore the genetic entity of this family. Results: A compound heterozygous missense mutation (NM_004793: c.2009C>T/p.A670V and c.2014C>T/p.R672C) of LONP1 was identified in the patient. Considering the clinical phenotypes and genetic results, the patient was diagnosed as CODAS syndrome. Conclusion: Here we reported the first case with CODAS syndrome in Chinese population. WES identified a compound heterozygous missense mutation of LONP1 gene in the patients. Our study not only provided data for genetic counseling and clinical diagnosis to this family, but also expanded the clinical spectrum of LONP1-related CODAS syndrome.

12.
Arch Biochem Biophys ; 710: 108983, 2021 10 15.
Artículo en Inglés | MEDLINE | ID: mdl-34228963

RESUMEN

Lon is an ATP-dependent protease belonging to the "ATPase associated with diverse cellular activities" (AAA+) protein family. In humans, Lon is translated as a precursor and imported into the mitochondria matrix through deletion of the first 114 amino acid residues. In mice, embryonic knockout of lon is lethal. In humans, some dysfunctional lon mutations are tolerated but they cause a developmental disorder known as the CODAS syndrome. To gain a better understanding on the enzymology of human mitochondrial Lon, this study compares the structure-function relationship of the WT versus one of the CODAS mutants R721G to identify the mechanistic features in Lon catalysis that are affected. To this end, steady-state kinetics were used to quantify the difference in ATPase and ATP-dependent peptidase activities between WT and R721G. The Km values for the intrinsic as well as protein-stimulated ATPase were increased whereas the kcat value for ATP-dependent peptidase activity was decreased in the R721G mutant. The mutant protease also displayed substrate inhibition kinetics. In vitro studies revealed that R721G did not degrade the endogenous mitochondrial Lon substrate pyruvate dehydrogenase kinase isoform 4 (PDK4) effectively like WT hLon. Furthermore, the pyruvate dehydrogenase complex (PDH) protected PDK4 from hLon degradation. Using hydrogen deuterium exchange/mass spectrometry and negative stain electron microscopy, structural perturbations associated with the R721G mutation were identified. To validate the in vitro findings under a physiologically relevant condition, the intrinsic stability as well as proteolytic activity of WT versus R721G mutant towards PDK 4 were compared in cell lysates prepared from immortalized B lymphocytes expressing the respective protease. The lifetime of PDK4 is longer in the mutant cells, but the lifetime of Lon protein is longer in the WT cells, which corroborate the in vitro structure-functional relationship findings.


Asunto(s)
Mitocondrias/enzimología , Proteasa La/química , Proteasa La/genética , Secuencia de Aminoácidos , Sustitución de Aminoácidos , Animales , Linfocitos B/enzimología , Biocatálisis , Anomalías Craneofaciales/enzimología , Anomalías Craneofaciales/genética , Estabilidad de Enzimas/genética , Anomalías del Ojo/enzimología , Anomalías del Ojo/genética , Trastornos del Crecimiento/enzimología , Trastornos del Crecimiento/genética , Luxación Congénita de la Cadera/enzimología , Luxación Congénita de la Cadera/genética , Humanos , Cinética , Ratones , Modelos Moleculares , Simulación de Dinámica Molecular , Proteínas Mutantes/química , Proteínas Mutantes/genética , Proteínas Mutantes/metabolismo , Mutación Missense , Osteocondrodisplasias/enzimología , Osteocondrodisplasias/genética , Proteasa La/metabolismo , Piruvato Deshidrogenasa Quinasa Acetil-Transferidora/metabolismo , Proteínas Recombinantes/química , Proteínas Recombinantes/genética , Proteínas Recombinantes/metabolismo , Relación Estructura-Actividad , Especificidad por Sustrato , Anomalías Dentarias/enzimología , Anomalías Dentarias/genética
13.
Clin Linguist Phon ; 35(6): 509-533, 2021 06 03.
Artículo en Inglés | MEDLINE | ID: mdl-32757784

RESUMEN

This study examined coda production in French-speaking children, aged 2;6 to 6;10 (n = 141). The primary aim was to provide normative information on coda production with a large group of children. The secondary aim was to investigate factors which influence coda production such as age, manner and place of articulation, word length, word position, and bilingualism. Children took part in a word-naming task in which they produced words containing word-final and -medial codas. Results indicated that French-speaking children, as young as 2;6, produce word-final codas with a high degree of accuracy (i.e. 80%). Age had minimal effects on coda presence (i.e., whether a coda was realized or not) but it did influence coda accuracy (i.e. whether a coda was realized target-like). Older children had better coda accuracy scores than younger children. Manner of articulation influenced coda production: the younger children had the lowest scores for liquid and fricative codas whereas the older children, for fricative codas. A closer examination of coda production according to voicing revealed it was voiced obstruent codas which obtained low accuracy scores across age groups. Word-length influenced coda realization with the youngest age group producing codas more often in one- versus two-syllable words. Children produced codas more often in word-final versus word-medial position with the greatest differences evident in the youngest children. Bilinguals obtained better coda scores than monolinguals at the youngest ages and poorer ones at the oldest ages. The study concludes with a discussion of the clinical implications of the findings.


Asunto(s)
Fonética , Voz , Adolescente , Niño , Lenguaje Infantil , Humanos , Lenguaje
14.
J Clin Med ; 9(6)2020 Jun 08.
Artículo en Inglés | MEDLINE | ID: mdl-32521756

RESUMEN

LONP1 is a nuclear-encoded mitochondrial protease crucial for organelle homeostasis; mutations of LONP1 have been associated with Cerebral, Ocular, Dental, Auricular, and Skeletal anomalies (CODAS) syndrome. To clarify the role of LONP1 in vivo, we generated a mouse model in which Lonp1 was ablated. The homozygous Lonp-/- mouse was not vital, while the heterozygous Lonp1wt/- showed similar growth rate, weight, length, life-span and histologic features as wild type. Conversely, ultrastructural analysis of heterozygous enterocytes evidenced profound morphological alterations of mitochondria, which appeared increased in number, swollen and larger, with a lower complexity. Embryonic fibroblasts (MEFs) from Lonp1wt/- mice showed a reduced expression of Lonp1 and Tfam, whose expression is regulated by LONP1. Mitochondrial DNA was also reduced, and mitochondria were swollen and larger, albeit at a lesser extent than enterocytes, with a perinuclear distribution. From the functional point of view, mitochondria from heterozygous MEF showed a lower oxygen consumption rate in basal conditions, either in the presence of glucose or galactose, and a reduced expression of mitochondrial complexes than wild type. In conclusion, the presence of one functional copy of the Lonp1 gene leads to impairment of mitochondrial ultrastructure and functions in vivo.

15.
Int Rev Cell Mol Biol ; 354: 1-61, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32475470

RESUMEN

Initially discovered as a protease responsible for degradation of misfolded or damaged proteins, the mitochondrial Lon protease (Lonp1) turned out to be a multifaceted enzyme, that displays at least three different functions (proteolysis, chaperone activity, binding of mtDNA) and that finely regulates several cellular processes, within and without mitochondria. Indeed, LONP1 in humans is ubiquitously expressed, and is involved in regulation of response to oxidative stress and, heat shock, in the maintenance of mtDNA, in the regulation of mitophagy. Furthermore, its proteolytic activity can regulate several biochemical pathways occurring totally or partially within mitochondria, such as TCA cycle, oxidative phosphorylation, steroid and heme biosynthesis and glutamine production. Because of these multiple activities, Lon protease is highly conserved throughout evolution, and mutations occurring in its gene determines severe diseases in humans, including a rare syndrome characterized by Cerebral, Ocular, Dental, Auricular and Skeletal anomalies (CODAS). Finally, alterations of LONP1 regulation in humans can favor tumor progression and aggressiveness, further highlighting the crucial role of this enzyme in mitochondrial and cellular homeostasis.


Asunto(s)
Proteasas ATP-Dependientes/metabolismo , Mitocondrias/enzimología , Proteínas Mitocondriales/metabolismo , Animales , Humanos , Ratones , Neoplasias/enzimología , Neoplasias/patología
16.
Waste Manag Res ; 38(6): 660-672, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31969081

RESUMEN

As the number of end-of-life vehicles (ELVs) increases rapidly, their management has become one of the most important environmental topics worldwide. This study is conducted to evaluate various alternatives for location selection of an authorized dismantling center (ADC) for ELVs using a multi-criteria decision-making (MCDM) approach. An intuitionistic fuzzy MCDM-based combinative distance-based assessment (CODAS) approach is proposed to aid waste managers and solve their problem. The intuitionistic fuzzy weighted averaging operator is utilized to aggregate individual opinions of decision-makers into a group opinion. The intuitionistic fuzzy Euclidean and Hamming distances are used to calculate the assessment score of alternatives. A real-life case study of Istanbul is provided to illustrate how this novel intuitionistic fuzzy MCDM-based CODAS approach can be used for alternative selection in real-world applications. The comparison with the available state-of-the-art intuitionistic fuzzy set-based MCDM approaches approves the validity and consistency of the proposed intuitionistic fuzzy CODAS approach. The intuitionistic fuzzy CODAS, WASPAS, and TOPSIS approaches generate exactly the same ordering of alternatives for the new ADC in Istanbul. The results show that the intuitionistic fuzzy CODAS approach indicates valid results and is an effective decision-making technique for vagueness and uncertainty nature of linguistic assessments.


Asunto(s)
Toma de Decisiones , Lógica Difusa , Incertidumbre
17.
Int J Mol Sci ; 20(18)2019 Sep 12.
Artículo en Inglés | MEDLINE | ID: mdl-31547314

RESUMEN

Research on healthy aging shows that lifespan reductions are often caused by mitochondrial dysfunction. Thus, it is very interesting that the deletion of mitochondrial matrix peptidase LonP1 was observed to abolish embryogenesis, while deletion of the mitochondrial matrix peptidase Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit (ClpP) prolonged survival. To unveil the targets of each enzyme, we documented the global proteome of LonP1+/- mouse embryonal fibroblasts (MEF), for comparison with ClpP-/- depletion. Proteomic profiles of LonP1+/- MEF generated by label-free mass spectrometry were further processed with the STRING (Search tool for the retrieval of interacting genes) webserver Heidelberg for protein interactions. ClpP was previously reported to degrade Eral1 as a chaperone involved in mitoribosome assembly, so ClpP deficiency triggers the accumulation of mitoribosomal subunits and inefficient translation. LonP1+/- MEF also showed Eral1 accumulation, but no systematic effect on mitoribosomal subunits. In contrast to ClpP-/- profiles, several components of the respiratory complex-I membrane arm, of the glutathione pathway and of lysosomes were accumulated, whereas the upregulation of numerous innate immune defense components was similar. Overall, LonP1, as opposed to ClpP, appears to have no effect on translational machinery, instead it shows enhanced respiratory dysfunction; this agrees with reports on the human CODAS syndrome (syndrome with cerebral, ocular, dental, auricular, and skeletal anomalies) caused by LonP1 mutations.


Asunto(s)
Proteasas ATP-Dependientes/metabolismo , Transporte de Electrón , Fibroblastos/metabolismo , Proteínas de Unión al GTP/metabolismo , Proteínas Mitocondriales/metabolismo , Ribosomas Mitocondriales/metabolismo , Proteínas de Unión al ARN/metabolismo , Animales , Células Cultivadas , Embrión de Mamíferos/citología , Embrión de Mamíferos/metabolismo , Ratones , Mapas de Interacción de Proteínas , Proteoma/metabolismo
18.
Data Brief ; 17: 237-245, 2018 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-29387738

RESUMEN

Seamounts are amongst the most common physiographic structures of the deep-ocean landscape, but remoteness and geographic complexity have limited the systematic collection of integrated and multidisciplinary data in the past. Consequently, important aspects of seamount ecology and dynamics remain poorly studied. We present a data collection of ocean currents and raw acoustic backscatter from shipboard Acoustic Doppler Current Profiler (ADCP) measurements during six cruises between 2004 and 2015 in the tropical and subtropical Northeast Atlantic to narrow this gap. Measurements were conducted at seamount locations between the island of Madeira and the Portuguese mainland (Ampère, Seine Seamount), as well as east of the Cape Verde archipelago (Senghor Seamount). The dataset includes two-minute ensemble averaged continuous velocity and backscatter profiles, supplemented by spatially gridded maps for each velocity component, error velocity and local bathymetry. The dataset is freely available from the digital data library PANGAEA at https://doi.pangaea.de/10.1594/PANGAEA.883193.

19.
Comput Ind Eng ; 119: 439-452, 2018 May.
Artículo en Inglés | MEDLINE | ID: mdl-32288046

RESUMEN

This paper aims to give deeper insights into decision making problem based on interval-valued fuzzy soft set (IVFSS). Firstly, a new score function for interval-valued fuzzy number is proposed for tackling the comparison problem. Subsequently, the formulae of information measures (distance measure, similarity measure and entropy) are introduced and their transformation relations are pioneered. Then, the objective weights of various parameters are determined via new entropy method, meanwhile, we develop the combined weights, which can show both the subjective information and the objective information. Moreover, we propose three algorithms to solve interval-valued fuzzy soft decision making problem by Weighted Distance Based Approximation (WDBA), COmbinative Distance-based ASsessment (CODAS) and similarity measure. Finally, the effectiveness and feasibility of approaches are demonstrated by a mine emergency decision making problem. The salient features of the proposed methods, compared to the existing interval-valued fuzzy soft decision making methods, are (1) it can obtain the optimal alternative without counterintuitive phenomena; (2) it has a great power in distinguishing the optimal alternative; and (3) it can avoid the parameter selection problems.

20.
Biochim Biophys Acta ; 1857(8): 1300-1306, 2016 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27033304

RESUMEN

Lon protease is a nuclear-encoded, mitochondrial ATP-dependent protease highly conserved throughout the evolution, crucial for the maintenance of mitochondrial homeostasis. Lon acts as a chaperone of misfolded proteins, and is necessary for maintaining mitochondrial DNA. The impairment of these functions has a deep impact on mitochondrial functionality and morphology. An altered expression of Lon leads to a profound reprogramming of cell metabolism, with a switch from respiration to glycolysis, which is often observed in cancer cells. Mutations of Lon, which likely impair its chaperone properties, are at the basis of a genetic inherited disease named of the cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome. This article is part of a Special Issue entitled 'EBEC 2016: 19th European Bioenergetics Conference, Riva del Garda, Italy, July 2-6, 2016', edited by Prof. Paolo Bernardi.


Asunto(s)
Anomalías Craneofaciales/genética , ADN Mitocondrial/genética , Anomalías del Ojo/genética , Trastornos del Crecimiento/genética , Luxación Congénita de la Cadera/genética , Mitocondrias/enzimología , Chaperonas Moleculares/química , Mutación , Osteocondrodisplasias/genética , Proteasa La/química , Anomalías Dentarias/genética , Reprogramación Celular , Anomalías Craneofaciales/enzimología , Anomalías Craneofaciales/patología , ADN Mitocondrial/metabolismo , Anomalías del Ojo/enzimología , Anomalías del Ojo/patología , Trastornos del Crecimiento/enzimología , Trastornos del Crecimiento/patología , Luxación Congénita de la Cadera/enzimología , Luxación Congénita de la Cadera/patología , Homeostasis , Humanos , Mitocondrias/patología , Modelos Moleculares , Chaperonas Moleculares/genética , Chaperonas Moleculares/metabolismo , Osteocondrodisplasias/enzimología , Osteocondrodisplasias/patología , Proteasa La/genética , Proteasa La/metabolismo , Pliegue de Proteína , Anomalías Dentarias/enzimología , Anomalías Dentarias/patología
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