Miracle-dz/UniFAR-FTUs huggingface dataset:
The effect of differentiating and apoptotic agents on notch signalling pathway in hepatoblastoma.

BACKGROUND/AIMS Notch expression is not yet determined in hepatoblastoma. In this study the effect of chemotherapeutics (cisplatin, doxorubicin, cytosin arabinoside); differentiating agent (13 cis-retinoic acid) and apoptotic agents (5-aza-2'-deoxycytidine, arsenic trioxide) on notch expression in hepatoblastoma were evaluated. METHODOLOGY After HepG2 cell line was cultured and the agents and their combinations were applied for 24 hour in pre-optimized 50% lethal doses, RNA isolation and cDNA converting, expression of 84 custom array genes of notch signaling pathway (SABiosciences, PAT059F-24) was determined by Real Time PCR. The methylation status of 6 genes that showed more than 5 fold changes compared with control group were explored by Methylation qPCR Assay. High expressed genes are HDAC1, NFKB1, CHUK, CDKN1A, and CBL. Low expressed genes are DLL1, CD44, FZD2, GLI1, IL17B, LMO2, NOTCH1, LOR, PAX5, PT-CRA, SH2D1A, and WISP1. The genes searched for methylation (DLL1, HEY1, DTX1, HDAC1, NOTCH2 and JAG1) were not found to be related with methylation. RESULTS The high expressed genes are related with cell proliferation. The main signaling genes that are closed to notch in signaling pathway are low expressed in hepatoblastoma. The agents do not show prominent effect of gene expression in many genes and methylation is not the reason of expression changes. The use of retinoic acid in the control of minimal residual disease of hepatoblastoma should be discussed. 5 aza "cytidin" the demethylating agent is not advised in treatment according to our results. CONCLUSION Cisplatin as main chemotherapeutic agent treatment is shown to change gene expression levels in notch signalling pathway in hepatoblastoma.
Three Tenets for Staged Correction of Kleeblattschädel or Cloverleaf Skull Deformity

Background: Patients with cloverleaf skull deformity are known to have high morbidity and poor outcome. Physical anomalies include a misshapen, trilobar skull, with a high “bossed” forehead, a bulging temporal region, and a flat posterior skull from multiple cranial suture fusion. Methods: Patients with cloverleaf skull deformity treated at the University of California, Los Angeles from 1990 to 2006 (n = 14) underwent early cranial vault remodeling (group 1) or staged correction with ventriculoperitoneal shunt (neonate), fronto-orbital advancement (3 to 6 months), and posterior vault remodeling (1 year) (group 2). Morbidity, necessary revisions, and neurologic (developmental testing) and aesthetic (Whitaker score) outcomes were assessed at follow-up. Results: Diagnoses included Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, Saethre-Chotzen syndrome, and nonsyndromic. Early cranial vault remodeling patients had more complications than staged correction patients [pneumonia, meningitis, and excessive bleeding (each 66 percent versus 9 percent); wound infection (66 percent versus 18 percent); and seizure (100 percent versus 0 percent)]. Early cranial vault remodeling patients had prolonged intensive care unit and hospital stays compared with staged correction patients (13 versus 2 days and 27 versus 5 days, respectively). The Whitaker score showed acceptable results at 18 months in group 2 (1.4, no revisions necessary) but not in group 1 (2.8, minor to major bony recontouring). Developmental tests showed that all early cranial vault remodeling patients had lower scores in both preschool tests and global evaluations compared with normative data (mean preschool receptive scores, 95 versus 85; mean preschool expressive scores, 94 versus 87). Conclusion: Staged correction of cloverleaf skull provided acceptable neurologic and aesthetic outcomes in the authors' series of patients.
Involvement of Endoplasmic Reticulum Stress in TULP1 Induced Retinal Degeneration

Inherited retinal disorders (IRDs) result in severe visual impairments in children and adults. A challenge in the field of retinal degenerations is identifying mechanisms of photoreceptor cell death related to specific genetic mutations. Mutations in the gene TULP1 have been associated with two forms of IRDs, early-onset retinitis pigmentosa (RP) and Leber congenital amaurosis (LCA). TULP1 is a cytoplasmic, membrane-associated protein shown to be involved in transportation of newly synthesized proteins destined for the outer segment compartment of photoreceptor cells; however, how mutant TULP1 causes cell death is not understood. In this study, we provide evidence that common missense mutations in TULP1 express as misfolded protein products that accumulate within the endoplasmic reticulum (ER) causing prolonged ER stress. In an effort to maintain protein homeostasis, photoreceptor cells then activate the unfolded protein response (UPR) complex. Our results indicate that the two major apoptotic arms of the UPR pathway, PERK and IRE1, are activated. Additionally, we show that retinas expressing mutant TULP1 significantly upregulate the expression of CHOP, a UPR signaling protein promoting apoptosis, and undergo photoreceptor cell death. Our study demonstrates that the ER-UPR, a known mechanism of apoptosis secondary to an overwhelming accumulation of misfolded protein, is involved in photoreceptor degeneration caused by missense mutations in TULP1. These observations suggest that modulating the UPR pathways might be a strategy for therapeutic intervention.
Quantitative studies of superior cervical sympathetic ganglia in a variety of primates including man. I. The ratio of preganglionic fibers to ganglionic neurons

Estimates of the number of ganglionic neurons of superior cervical sympathetic ganglia and the number of preganglionic axons in the trunks just caudal to these ganglia were obtained from a sample of primates that included: man, chimpanzee, baboon, stump‐tailed macaque, rhesus monkey, and squirrel monkey. The number of ganglionic neurons ranged from 63,625 in a squirrel monkey ganglion to 1,041,652 neurons in a human ganglion. Estimates of the number of preganglionic fibers varied between 2,285 in a cervical sympathetic trunk of a squirrel monkey and 12,008 in a human specimen. The resulting ratios of preganglionic fibers to ganglionic neurons ranged from 1:28 in a squirrel monkey ganglion to 1:196 in a human ganglion.
Developmental dysplasia of the hip.

1. Blaise A. Nemeth, MD, MS* 2. Vinay Narotam, MD† 1. *Associate Professor, Department of Orthopedics and Rehabilitation, University of Wisconsin School of Medicine and Public Health, Madison, WI. 2. †Assistant Professor, Department of Orthopaedics, University of North Carolina School of Medicine, Chapel Hill, NC. * Abbreviations: AVN: : avascular necrosis DDH: : developmental dysplasia of the hip Early detection of developmental dysplasia of the hip is essential because restoration of the normal relationship of the femoral head and acetabulum increases the likelihood of normal development. Pediatricians must be aware of the American Academy of Pediatrics guidelines for early detection. After completing this article, readers should be able to: 1. Acknowledge the spectrum of hip pathology included in developmental dysplasia of the hip (DDH). 2. Identify newborns at risk for DDH. 3. Diagnose hip dislocations by using appropriate physical examination maneuvers. 4. Appropriately use imaging modalities to screen for DDH in infants who have normal or equivocal physical findings. 5. Recognize the presentation of hip dislocation in the older child. Developmental dysplasia of the hip (DDH) encompasses the spectrum of hip abnormalities involving the relationship between the femoral head and the acetabulum during early growth and development. A hip may be dislocated at rest, dislocatable (but in a normal position at rest), subluxed (incomplete contact between the femoral head and acetabulum), subluxable (incomplete contact induced with provocative maneuvers), or appear normal on physical examination yet have an abnormally shaped acetabulum or femoral head radiographically. The previously used term, “congenital hip dislocation,” has been abandoned in recognition of this spectrum, acknowledging as well the fact that a child may have normal examination findings at birth but progress to dislocation later in life. Strictly speaking, the term DDH does not apply to abnormal development of the hip due to other diseases, such as cerebral palsy, Legg-Calve-Perthes disease, or slipped capital femoral epiphysis, in which “hip dysplasia” is a sufficient term, nor does the term include traumatic dislocation. In addition, the term “teratologic dislocation” is reserved …
Proapoptotic BAX and BAK Modulate the Unfolded Protein Response by a Direct Interaction with IRE1α

Accumulation of misfolded protein in the endoplasmic reticulum (ER) triggers an adaptive stress response—termed the unfolded protein response (UPR)—mediated by the ER transmembrane protein kinase and endoribonuclease inositol-requiring enzyme–1α (IRE1α). We investigated UPR signaling events in mice in the absence of the proapoptotic BCL-2 family members BAX and BAK [double knockout (DKO)]. DKO mice responded abnormally to tunicamycin-induced ER stress in the liver, with extensive tissue damage and decreased expression of the IRE1 substrate X-box–binding protein 1 and its target genes. ER-stressed DKO cells showed deficient IRE1α signaling. BAX and BAK formed a protein complex with the cytosolic domain of IRE1α that was essential for IRE1α activation. Thus, BAX and BAK function at the ER membrane to activate IRE1α signaling and to provide a physical link between members of the core apoptotic pathway and the UPR.
Perceptions of world benevolence, meaningfulness, and self-worth among elderly israeli holocaust survivors and non-survivors

It has been hypothesized that traumatic events may challenge and even disrupt basic individual assumptions about the world, including the perception of the world as a benevolent place, the meaningfulness of the world, and the self-worth of the individual. The present study compared the cognitive schemata of 61 Israeli Holocaust survivors and 131 controls. Multivariate analysis of variance indicated a significant overall Holocaust effect on World Assumptions. Univariate F tests implicated two of the three schemata investigated: world benevolence and world meaning. Exposure to the Holocaust accounted for almost all the explained variance in the world benevolence scheme, while sociodemographic variables contributed almost all of the explained variance to world meaning and self-worth. The theoretical implication of the results are discussed.
Differentiation of germ cells and gametes from stem cells.

BACKGROUND Advances in stem cell research have opened new perspectives for regenerative and reproductive medicine. Stem cells (SC) can differentiate under appropriate in vitro and in vivo conditions into different cell types. Several groups have reported their ability to differentiate SCs into germline cells, and some of them have been successful in obtaining male and female gamete-like cells by using different methodologies. METHODS This review summarizes the current knowledge in this field and emphasizes significant embryological, genetic and epigenetic aspects of germ cells and gametes in vitro differentiation in humans and other species, highlighting major obstacles that need to be overcome for successful gametogenesis in culture: studies reporting development of germ cell-like cells from murine and human embryonic (ESC) and somatic SCs are critically reviewed. RESULTS Published studies indicate that germ cells can be consistently differentiated from mouse and human ESC. However, further differentiation of germ cells through gametogenesis still has important genetic and epigenetic obstacles to be efficient. CONCLUSIONS Differentiation of germ cells from SCs has the potential of becoming a future source of gametes for research use, although further investigation is needed to understand and develop the appropriate niches and culture conditions. Additionally, if genetic and epigenetic methodological limitations could be solved, therapeutic opportunities could be also considered.
Reading Korean Public Administration: An Application of Lacanian Four Discourses

Abstract The article attempts to make a discourse analysis on Korean public administration research for the past half century, in terms of Jacque Lacan's theory of the four fundamental discourses. Of the four discourses, the master discourse and university discourse are dominant, while the analytic discourse and hysteric discourse are marginalized. Previous studies have served or justified the three master signifiers in Korean public administration (the hierarchical political power, capitalism, and the U.S. public administration), with little attempt to challenge and change those master signifiers. Some structured mechanisms, like the hierarchical structure of governance and the tripartite disciplinary network, have contributed to the discourse (re)production. The article argues for making efforts to dissolve those reproduction mechanisms, and for a change of discourses in Korean public administration research.
The Prevalence of Demodex Species and Its Relationship With the Metabolic Syndrome in Women of Malatya Province, Turkey

Background: Demodex species are ectoparasites living in the hair follicles and sebaceous glands in human. Only two species, Demodex folliculorum and D. brevis were identified in human. While the D. folliculorum is settling in infundibular part of the hair follicles mostly, D. brevis settles into the sebaceous glands and ducts, which are deeper. These parasites live preferentially in hair follicles on the face and in the sebaceous glands, although they have also been reported to reside in seborrheic parts of the human body. The Demodex species have the highest rate on the face which has thesignificant number of sebaceous glands and sebum production in the skin. However, the rate of infestation increases with age in healthy skin. Objectives: The aim of this study was to determine the prevalence of Demodex species in healthy women and the relationship between the incidence of Demodex and metabolic syndrome (MetS). Patients and Methods: This study consisted of 151,498 women aged ≥ 20 years who reside in the central district of Malatya province, Turkey. In 5% confidence interval of sample size, while the design effect was 1.5 it was calculated as 552 individuals and while the design effect was 2 it was calculated as 736 individuals. The World Health Organization 30 cluster sampling method was used to select the samples. Women aged ≥ 20 years who were not pregnant or lactating were included in the study. From a total of 669 subjects included in this study, 90.89% of the largest sample was accessed. Results: Parasites were detected in 263 (39.3%) of 669 subjects and 3 of them were D. brevis. In chi-square analysis, nosignificant relationship was found between the incidence of the parasite, age, education level, occupation, marital status, family type, and MetS. However, a significant relationship was found between the diastolic pressure and those who fed with fatty foods and the incidence of parasite’s occurrence. Conclusions: According to the results of this study, MetS has no effect on the frequency of occurrence of the parasite; however, weight, fatty foods, and high diastolic pressure are effective in the frequency of occurrence of the parasite. The effects of these factors on the incidence of parasites should be supported by further study designs.
Heterogeneous-race-free memory models

Commodity heterogeneous systems (e.g., integrated CPUs and GPUs), now support a unified, shared memory address space for all components. Because the latency of global communication in a heterogeneous system can be prohibi-tively high, heterogeneous systems (unlike homogeneous CPU systems) provide synchronization mechanisms that only guarantee ordering among a subset of threads, which we call a scope. Unfortunately, the consequences and se-mantics of these scoped operations are not yet well under-stood. Without a formal and approachable model to reason about the behavior of these operations, we risk an array of portability and performance issues. In this paper, we embrace scoped synchronization with a new class of memory consistency models that add scoped synchronization to data-race-free models like those of C++ and Java. Called sequential consistency for heterogeneous-race-free (SC for HRF), the new models guarantee SC for programs with "sufficient" synchronization (no data races) of "sufficient" scope. We discuss two such models. The first, HRF-direct, works well for programs with highly regular parallelism. The second, HRF-indirect, builds on HRF-direct by allowing synchronization using different scopes in some cases involving transitive communication. We quanti-tatively show that HRF-indirect encourages forward-looking programs with irregular parallelism by showing up to a 10% performance increase in a task runtime for GPUs.
Increased expression of membrane-type matrix metalloproteinase and preferential localization of matrix metalloproteinase-2 to the neointima of balloon-injured rat carotid arteries.

BACKGROUND Remodeling of the injured vascular wall is dependent on the action of several extracellular proteases. Previous studies have shown that expression of matrix metalloproteinases (MMP-2 and MMP-9) is upregulated after vascular injury and that MMP-2 is required for the migration of cultured vascular smooth muscle cells across complex extracellular matrix barriers. The present study examined changes in the expression of membrane-type metalloproteinase (MT-MMP-1), a putative regulator of MMP-2, in the tissue localization of MMP-2, and in the expression of activated and latent forms of MMP-2 and the tissue inhibitor of metalloproteinases, TIMP-2, in rat carotid arteries subjected to balloon catheter injury. METHODS AND RESULTS MT-MMP-1 mRNA levels increased sixfold after 3 days of injury, coinciding with an increase in MMP-2 activation assessed by gelatin zymography. Western blotting and gelatin zymography showed an increase in MMP-2 protein levels beginning 5 to 7 days after injury; immunocytochemistry and Western blotting showed that the increase occurred preferentially in the developing neointima. CONCLUSIONS These results show that increased expression of MT-MMP-1 and activation of MMP-2 occurs early after injury to the rat carotid artery and that at later times MMP-2 is preferentially localized to the developing neointima.
Beyond the Point: A Basic Guide to Literature on Pointing Abilities in Children with Autism

Research on core behavioral features of autism spectrum disorder (ASD) has always been a challenging endeavor. Amongst these features pointing abilities have often attracted attention of researchers. Traditional studies on pointing tended to rely mostly on the distinction between imperative and declarative pointing, but research has gradually recognized the importance of developmental trajectories and the relevance of other skills in the acquisition of pointing in children with ASD. The present study aims to offer a basic review of the literature on pointing in children with ASD, in order to explain these changes in research focus and to pinpoint relevant aspects of pointing that have emerged across time which may be of relevance for future studies.
The Impact of Completeness and Web Use Motivation on the Credibility of e‐Health Information

Recent articles on the quality of health information on the Internet reveal 2 critical criteria: completeness and credibility. This article investigates the effect of Web use motivation on the relationship between completeness and consumer perceptions of credibility. Based on a 2 × 3 experiment conducted with 246 respondents, the article demonstrates that the extent of completeness of health information on the Internet impacts consumer assessment of source and website credibility. In contrast to the extant research on the orthogonality of content and source characteristics, this research demonstrates their interaction.
Families with Children Who Have Autism Spectrum Disorders: Stress and Support

Many individuals with autism spectrum disorders (ASD) have behavior repertoires that might be expected to have an impact on members of both the immediate and extended family. This article examines this impact, reviewing relevant literature related to stressors and supports for families of individuals with ASD. The focus of the article highlights research on stressors and supports in the following areas (a) stress in the marital subsystem, (b) stress in the parental subsystem, (c) stress in the sibling subsystem, (d) coping strategies used by families, and (e) informal and formal sources of support used by families. Implications and recommendations for future research and practice are discussed.
Overprvning Large Decision Trees

This paper presents empirical evidence for five hypotheses about learning from large noisy domains: that trees built from very large training sets are larger and more accurate than trees built from even large subsets; that this increased accuracy is only in part due to the extra size of the trees; and that the extra training instances allow both better choices of attribute while building the tree, and better choices of the subtrees to prune after it has been built. For the practitioner with the common goals of maximising the accuracy and minimising the size of induced trees, these conclusions prompt new techniques for induction on large training sets. Although building huge trees from huge training sets is computationally expensive, pruning smaller trees on them is not, yet it improves accuracy. Where a pruned tree is considered too large for human or machine limitations, it can be overpruncd to an acceptable size. Although this requires far more time than building a tree of that size from a correspondingly small training set, it wi l l usually be more accurate. The paper also describes an algorithm for overpruning trees to user-specified size limits; it is evaluated in the course of testing the above hypotheses.
The mechanical function of linked muscles in the guinea fowl hind limb

SUMMARY Although mechanical linkages between the proximal and distal limb are present in a range of species, their functional significance is unknown. We have investigated the mechanical function of the flexor cruris lateralis pars pelvica (FCLP), flexor cruris lateralis pars accessoria (FCLA) and gastrocnemius intermedia (GI), a system of linked muscles spanning proximal and distal limb segments in the guinea fowl (Numida meleagris) hind limb. The FCLP, which is in the anatomical position of a hamstring muscle, is the primary component of the linkage. It is connected to the distal femur via the FCLA, the tarsometatarsus via the tendon of insertion of the GI and the common Achilles tendon, and the tibiotarsus via a distal tendon of insertion. The FCLP may, therefore, potentially exert moments at the hip, knee and ankle joints depending on the joint angles and the relative states of activation in the three muscles. Evidence presented here suggests that the GI and FCLA act as actively controlled links that alter distal action of the FCLP. The FCLP and GI are coactive in the late swing and early stance phases of the stride, forming a triarticular complex, and likely act together to resist and control ankle flexion immediately after foot-down in addition to providing hip extension and knee flexion moments. The FCLP and FCLA are coactive from mid-through to late stance, acting together as a uniarticular hip extensor. Available evidence suggests that this role of the FCLP and FCLA is of increased importance in inclined running and accelerations. This linkage between a proximal muscle and alternate distal connections allows for functional flexibility, both in terms of the site at which the muscle exerts force and the nature of the muscle's mechanical function. The interactions generated between the proximal and distal limb by linkages of this type suggest that less emphasis should be placed on the distinct functional roles of specific anatomical classes of muscle within proximal and distal limb segments.