The Oak Ridge Polycystic Kidney mouse: Modeling ciliopathies of mice and men

The Oak Ridge Polycystic Kidney (ORPK) mouse was described nearly 14 years ago as a model for human recessive polycystic kidney disease. The ORPK mouse arose through integration of a transgene into an intron of the Ift88 gene resulting in a hypomorphic allele (Ift88(Tg737Rpw)). The Ift88(Tg737Rp omega) mutation impairs intraflagellar transport (IFT), a process required for assembly of motile and immotile cilia. Historically, the primary immotile cilium was thought to have minimal importance for human health; however, a rapidly expanding number of human disorders have now been attributed to ciliary defects. Importantly, many of these phenotypes are present and can be analyzed using the ORPK mouse. In this review, we highlight the research conducted using the OPRK mouse and the phenotypes shared with human cilia disorders. Furthermore, we describe an additional follicular dysplasia phenotype in the ORPK mouse, which alongside the ectodermal dysplasias seen in human Ellis-van Creveld and Sensenbrenner's syndromes, suggests an unappreciated role for primary cilia in the skin and hair follicle

Relationship among expression of basic-fibroblast growth factor, MTDH/Astrocyte elevated gene-1, adenomatous polyposis coli, matrix metalloproteinase 9,and COX-2 markers with prognostic factors in prostate carcinomas

Background: The etiopathogenesis of prostate cancer (PC) is still not clear, but hormonal, genetic, and environmental factors are thought to play a role in the tumor pathogenesis. Astrocyte elevated gene-1(AEG-1) as a novel transmembrane protein is predominantly located in the perinuclear region and endoplasmic reticulum. It has been found that AEG-1 upregulation increases the invasive ability of glioma and prostate cancer. Basic fibroblast growth factor (bFGF), matrix metalloproteinase-9 (MMP-9), cyclooxygenases-2 (COX-2), and adenomatous polyposis coli (APC) are very important in tumor progression as well. Materials and Methods: This study included 97 radical prostatectomy specimens. IHC stains for bFGF, MMP-9, COX-2, APC, and AEG-1 were performed on the tissue microarray using standard procedures. For each patient, the age, Gleason score, tumor volume, lymphovascular invasion, lymph node metastasis, surgical margin, and the invasion of vesiculoseminalis areas were assessed. Analyses were performed using the statistical PASW (ver. 18). Results: Statistically significant positive relationships were found MMP-9 and COX-2 (r = 0.242 and P = 0.017), between MMP-9 and APC (r = 0.207 and P = 0.043), and between bFGF and AEG-1 (r = 0.295 and P = 0.004). However, the relationships between age and staining results and tumor volume and staining results were not found to be significant. Although a positive correlation was found between the Gleason score and tumor volume and the Gleason score and age (r = 0.415 and P = 0.0001; r = 0.246 and P = 0.015, respectively), we did not find a statistically significant relationship between other stains and other prognostic parameters (lymphovascular invasion, lymph node metastasis, surgical margin, or vesiculoseminalis invasion). Conclusion:The relationships we found between MMP-9 and COX-2, between MMP-9, and APC and between bFGF and AEG-1 as independent prognostic parameters could be helpful in the development of new therapeutic procedures.Keywords: Adenomatous polyposis coli, astrocyte elevated gene‑1, basic fibroblast growth factor, cyclooxygenases‑2, matrix metalloproteinase‑9, prognostic parameters, prostate adenocarcinomasNigerian Journal of Clinical Practice • Oct-Dec 2013 • Vol 16 • Issue

Foreign direct investment, stock market capitalization, and sustainable development: relative impacts of domestic and foreign capital

It is well acknowledged that achieving sustainable development goals without negatively impacting a country’s economic activity is complicated. The question of whether foreign or domestic capital can be used to address the financial demands of the nations who lack the financial resources for a green transformation should now be resolved. Based on this, the main goal of this research is to analyze the impacts of domestic and foreign capital on carbon emissions for a heterogeneous panel of 42 countries for the period from 1990 to 2017. Aside from capital accumulation, the environmental impact of elements such as economic growth, urbanization, trade openness, and energy usage is also studied. The newly developed quantile via moment approach is utilized to isolate the impacts according to the countries’ emission levels. Finally, the impact of these variables on the recently constructed sustainable development index is investigated in order to ensure its robustness. The findings of the study reveal that the environmental efficiency of domestic capital accumulation in countries with low emission levels is higher than in countries with high emission levels. Foreign capital, on the other hand, has no substantial effect on emission levels in all quantiles. © 2022, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature

Capital Fixity and Mobility in Response to the 2008-09 Crisis: Variegated Neoliberalism in Mexico and Turkey

The article examines the 2008-9 crisis responses in Mexico and Turkey as examples of variegated neoliberalism. The simultaneous interests of corporations and banks relative to the national fixing of capital and their mobility in the form of global investment heavily influenced each state authority’s policy responses to the crisis at the expense of the interests of the poor, workers, and peasantry. Rather than pitching this as either evidence of persistent national differentiation or some Keynesian state resurgence, we argue from a historical materialist geographical framework that the responses of capital and state authorities in Mexico and Turkey actively constitute and reconstitute the global parameters of market regulatory design and neoliberal class rule through each state’s distinct domestic policy formation and crisis management processes. While differing in specific content the form of Mexico and Turkey’s state responses to the crisis ensured continuity in their foregoing neoliberal strategies of development and capital accumulation, most notably in the continued oppression of workers. That is, the prevailing strategy of accumulation continues to be variegated neoliberalism

New features for child metrics: Further growth references and blood pressure calculations

Many new features have recently been incorporated to CEDD Cozum/Child Metrics, an online and freely accessible scientific toolset. Various auxological assessments can now be made with data of children with genetic diseases (Prader Willi syndrome, Noonan syndrome, Turner syndrome, Down syndrome, and Achondroplasia) and preterm and term newborns. More detailed reports for height, weight, and body mass index data of a given child are now available. Last but not least, office and 24-hour ambulatory blood pressure values can be analyzed according to normative data

Analytical, numerical, and experimental investigation of a Luneburg lens system for directional cloaking

In this study, the design of a directional cloaking based on the Luneburg lens system is proposed and its operating principle is experimentally verified. The cloaking concept is analytically investigated via geometrical optics and numerically realized with the help of the finite-difference time-domain method. In order to benefit from its unique focusing and/or collimating characteristics of light, the Luneburg lens is used. We show that by the proper combination of Luneburg lenses in an array form, incident light bypasses the region between junctions of the lenses, i.e., the "dark zone." Hence, direct interaction of an object with propagating light is prevented if one places the object to be cloaked inside that dark zone. This effect is used for hiding an object which is made of a perfectly electric conductor material. In order to design an implementable cloaking device, the Luneburg lens is discretized into a photonic crystal structure having gradually varying air cylindrical holes in a dielectric material by using Maxwell Garnett effective medium approximations. Experimental verifications of the designed cloaking structure are performed at microwave frequencies of around 8 GHz. The proposed structure is fabricated by three-dimensional printing of dielectric polylactide material and a brass metallic alloy is utilized in place of the perfectly electric conductor material in microwave experiments. Good agreement between numerical and experimental results is found. © 2019 American Physical Society

Turner syndrome and associated problems in turkish children: A multicenter study

Objective: Turner syndrome (TS) is a chromosomal disorder caused by complete or partial X chromosome monosomy that manifests various clinical features depending on the karyotype and on the genetic background of affected girls. This study aimed to systematically investigate the key clinical features of TS in relationship to karyotype in a large pediatric Turkish patient population. Methods: Our retrospective study included 842 karyotype-proven TS patients aged 0-18 years who were evaluated in 35 different centers in Turkey in the years 2013-2014. Results: The most common karyotype was 45,X (50.7%), followed by 45,X/46,XX (10.8%), 46,X,i(Xq) (10.1%) and 45,X/46,X,i(Xq) (9.5%). Mean age at diagnosis was 10.2±4.4 years. The most common presenting complaints were short stature and delayed puberty. Among patients diagnosed before age one year, the ratio of karyotype 45,X was significantly higher than that of other karyotype groups. Cardiac defects (bicuspid aortic valve, coarctation of the aorta and aortic stenosi) were the most common congenital anomalies, occurring in 25% of the TS cases. This was followed by urinary system anomalies (horseshoe kidney, double collector duct system and renal rotation) detected in 16.3%. Hashimoto’s thyroiditis was found in 11.1% of patients, gastrointestinal abnormalities in 8.9%, ear nose and throat problems in 22.6%, dermatologic problems in 21.8% and osteoporosis in 15.3%. Learning difficulties and/or psychosocial problems were encountered in 39.1%. Insulin resistance and impaired fasting glucose were detected in 3.4% and 2.2%, respectively. Dyslipidemia prevalence was 11.4%. Conclusion: This comprehensive study systematically evaluated the largest group of karyotype-proven TS girls to date. The karyotype distribution, congenital anomaly and comorbidity profile closely parallel that from other countries and support the need for close medical surveillance of these complex patients throughout their lifespan. © Journal of Clinical Research in Pediatric Endocrinology

Returns to Foreign Language Skills in a Developing Country: The Case of Turkey

Foreign language skills represent a form of human capital that can be rewarded in the labor market. Drawing on data from the Adult Education Survey of 2007, this is the first study estimating returns to foreign language skills in Turkey. We contribute to the literature on the economic value of language knowledge, with a special focus on a country characterized by fast economic and social development. Although English is the most widely spoken foreign language in Turkey, we initially consider the economic value of different foreign languages among the employed males aged 25 to 65. We find positive and significant returns to proficiency in English and Russian, which increase with the level of competence. Knowledge of French and German also appears to be positively rewarded in the Turkish labor market, although their economic value seems mostly linked to an increased likelihood to hold specific occupations rather than increased earnings within occupations. Focusing on English, we also explore the heterogeneity in returns to different levels of proficiency by frequency of English use at work, birth-cohort, education, occupation and rural/urban location. The results are also robust to the endogenous specification of English language skills

The walls of gated communities in Brazil and Turkey: security, separation or status?

Gated communities are common types of housing estates found in many countries. They are based on strictly controlled access of people. Houses, and at times high-rise apartment blocks, are built behind a common wall, along private internal streets. Both in Brazil and in Turkey these types of residential neighborhoods can be found mainly on the outskirts of large urban areas. This paper investigates the reasons for the increase of such residential areas, in relation to the two countries urban problems. In Brazil the dominant reason for the proliferation of gated communities, found in the literature and in advertisement of such estates, is security, in view of the countries high crime rates. In Turkey the main reason for a family to choose to live in such residential areas is status and privacy. Although Brazil and Turkey have very different cultural backgrounds, in both countries gated communities are increasingly popular. The attraction of these so-called communities must therefore be analyzed. Are people more vulnerable in large mega-cities? Also, the impact on urban prospects as a whole must be discussed. Socio-cultural and psychological concepts such as territoriality, security, privacy, which can be represented by a pattern of behavior of an individual or group, as based on control of space, are thus touched on in the paper. Conclusions confirm that the reasons for preferences for gated communities are the feeling of belonging to a special place, fear of crime and a sense security and determine decision making of families in their homeownership choices