Factors related to seat belt use : A Turkish case

Seat belts are effective safety devices used to protect car occupants from severe injuries and fatalities during road vehicle accidents. Despite the proven effectiveness of seat belts, seat belt use rates are quite low, especially in developing countries, such as Turkey. The general aim of the present study was to investigate a large variety of factors related to seat belt use among Turkish car occupants using different perspectives and methods and therefore, to contribute to the design of effective seat belt use interventions for increasing seat belt use rates in Turkey. Five sub-studies were conducted within the present study. In the first sub-study, environmental (e.g., road type) and psycho-social factors (e.g., belt use by other car occupants) related to the seat belt use of front-seat occupants were investigated using observation techniques. Being male, of a young age, and traveling on city roads were the main factors negatively related to seat belt use. Furthermore, seat belt use by the drivers and front-seat passengers was highly correlated and a significant predictors of each other. In the second sub-study, the motivations of the car occupants for seat belt use and non-use were investigated using interview techniques. Situational conditions, such as traveling on city roads and for short distances, and not believing in the effectiveness and relevance of seat belt use for safety, were the most frequently reported reasons for not using a seat belt. Safety, habit and avoiding punishment were among the most frequently reported reasons for using a seat belt. In the third sub-study, the Theory of Planned Behavior (TPB) and the Health Belief Model (HBM) were applied to seat belt use using Structural Equation Modeling techniques. The TPB model showed a good fit to the data, whereas the HBM showed a poor fit to the data. Within the TPB model, attitude and subjective norm were significant predictors of intentions to use a seat belt on both urban and rural roads. In the fourth sub-study, seat belt use frequency and motivations for seat belt use among taxi drivers were investigated and compared between free-time and work-time driving using a survey. The results showed that taxi drivers used seat belts more when driving a private car in their free-times compared to when driving a taxi during their work-times. The lack of a legal obligation to use a seat belt in city traffic and fear of being attacked or robbed by the passengers were found as two specific reasons for not using a seat belt when driving a taxi. Lastly, in the fifth sub-study, the relationship of seat belt use to driver and health behaviors was investigated using a survey. Although seat belt use was related both to health and driver behaviors, factor analysis results showed that it grouped with driver behaviors. Based on the results of the sub-studies, a tentative empirical model showing different predictors of seat belt use was proposed. According to the model, safety and normative motivations and perceived physical barriers related to seat belt use are the three important predictors of seat belt use. Keywords: Seat belt use; environmental factors; psycho-social factors; safety and normative motivations; the Theory of Planned Behavior; the Health Belief Model; health behaviors; driver behaviors; front-seat occupants; taxi drivers; Turkey.

The regional differences between countries in traffic safety: A cross-cultural study and Turkish Case

Road traffic accidents are a large problem everywhere in the world. However, regional differences in traffic safety between countries are considerable. For example, traffic safety records are much worse in Southern Europe and the Middle East than in Northern and Western Europe. Despite the large regional differences in traffic safety, factors contributing to different accident risk figures in different countries and regions have remained largely unstudied. The general aim of this study was to investigate regional differences in traffic safety between Southern European/Middle Eastern (i.e., Greece, Iran, Turkey) and Northern/Western European (i.e., Finland, Great Britain, The Netherlands) countries and to identify factors related to these differences. We conducted seven sub-studies in which I applied a traffic culture framework, including a multi-level approach, to traffic safety. We used aggregated level data (national statistics), surveys among drivers, and data on traffic accidents and fatalities in the analyses. In the first study, we investigated the influence of macro level factors (i.e., economic, societal, and cultural) on traffic safety across countries. The results showed that a high GNP per capita and conservatism correlated with a low number of traffic fatalities, whereas a high degree of uncertainty avoidance, neuroticism, and egalitarianism correlated with a high number of traffic fatalities. In the second, third, and fourth studies, we examined whether the conceptualisation of road user characteristics (i.e., driver behaviour and performance) varied across traffic cultures and how these factors determined overall safety, and the differences between countries in traffic safety. The results showed that the factorial agreement for driver behaviour (i.e., aggressive driving) and performance (i.e., safety skills) was unsatisfactory in Greece, Iran, and Turkey, where the lack of social tolerance and interpersonal aggressive violations seem to be important characteristics of driving. In addition, we found that driver behaviour (i.e., aggressive violations and errors) mediated the relationship between culture/country and accidents. Besides, drivers from "dangerous" Southern European countries and Iran scored higher on aggressive violations and errors than did drivers from "safe" Northern European countries. However, "speeding" appeared to be a "pan-cultural" problem in traffic. Similarly, aggressive driving seems largely depend on road users' interactions and drivers' interpretation (i.e., cognitive biases) of the behaviour of others in every country involved in the study. Moreover, in all countries, a risky general driving style was mostly related to being young and male. The results of the fifth and sixth studies showed that among young Turkish drivers, gender stereotypes (i.e., masculinity and femininity) greatly influence driver behaviour and performance. Feminine drivers were safety-oriented whereas masculine drivers were skill-oriented and risky drivers. Since everyday driving tasks involve not only erroneous (i.e., risky or dangerous driving) or correct performance (i.e., normal habitual driving), but also "positive" driver behaviours, we developed a reliable scale for measuring "positive" driver behaviours among Turkish drivers in the seventh study. Consequently, I revised Reason's model [Reason, J. T., 1990. Human error. Cambridge University Press: New York] of aberrant driver behaviour to represent a general driving style, including all possible intentional behaviours in traffic while evaluating the differences between countries in traffic safety. The results emphasise the importance of economic, societal and cultural factors, general driving style and skills, which are related to exposure, cognitive biases as well as age, sex, and gender, in differences between countries in traffic safety.

Preadolescents and Their Mothers as Oral Health-Promoting Actors : Non-biologic Determinants of Oral Health among Turkish and Finnish Preadolescents

Is oral health becoming a part of the global health culture? Oral health seems to turn out to be part of the global health culture, according to the findings of a thesis-research, Institute of Dentistry, University of Helsinki. The thesis is entitled as “Preadolescents and Their Mothers as Oral Health-Promoting Actors: Non-biologic Determinants of Oral Health among Turkish and Finnish Preadolescents.” The research was supervised by Prof.Murtomaa and led by Dr.A.Basak Cinar. It was conducted as a cross-sectional study of 611 Turkish and 223 Finnish school preadolescents in Istanbul and Helsinki, from the fourth, fifth, and sixth grades, aged 10 to 12, based on self-administered and pre-tested health behavior questionnaires for them and their mothers as well as the youth’s oral health records. Clinically assessed dental status (DMFT) and self-reported oral health of Turkish preadolescents was significantly poorer than the Finns`. A similar association occurred for well-being measures (height and weight, self-esteem), but not for school performance. Turkish preadolescents were more dentally anxious and reported lower mean values of toothbrushing self-efficacy and dietary self-efficacy than did Finns. The Turks less frequently reported recommended oral health behaviors (twice daily or more toothbrushing, sweet consumption on 2 days or less/week, decreased between-meal sweet consumption) than did the Finns. Turkish mothers reported less frequently dental health as being above average and recommended oral health behaviors as well as regular dental visits. Their mean values for dental anxiety was higher and self-efficacy on implementation of twice-daily toothbrushing were lower than those of the Finnish. Despite these differences between the Turks and Finns, the associations found in common for all preadolescents, regardless of cultural differences and different oral health care systems, assessed for the first time in a holistic framework, were as follows: There seems to be interrelation between oral health and general-well being (body height-weight measures, school performance, and self-esteem) among preadolescents: • The body height was an explanatory factor for dental health, underlining the possible common life-course factors for dental health and general well-being. • Better school performance, high levels of self-esteem and self-efficacy were interrelated and they contributed to good oral health. • Good school performance was a common predictor for twice-daily toothbrushing. Self-efficacy and maternal modelling have significant role for maintenance and improvement of both oral- and general health- related behaviors. In addition, there is need for integration of self-efficacy based approaches to promote better oral health. • All preadolescents with high levels of self-efficacy were more likely to report more frequent twice-daily toothbrushing and less frequent sweet consumption. • All preadolescents were likely to imitate toothbrushing and sweet consumption behaviors of their mothers. • High levels of self-efficacy contributed to low dental anxiety in various patterns in both groups. As a conclusion: • Many health-detrimental behaviors arise from the school age years and are unlikely to change later. Schools have powerful influences on children’s development and well-being. Therefore, oral health promotion in schools should be integrated into general health promotion, school curricula, and other activities. • Health promotion messages should be reinforced in schools, enabling children and their families to develop lifelong sustainable positive health-related skills (self-esteem, self-efficacy) and behaviors. • Placing more emphasis on behavioral sciences, preventive approaches, and community-based education during undergraduate studies should encourage social responsibility and health-promoting roles among dentists. Attempts to increase general well-being and to reduce oral health inequalities among preadolescents will remain unsuccessful if the individual factors, as well as maternal and societal influences, are not considered by psycho-social holistic approaches.

Towards Multiculturalism in Turkish Public Service Broadcasting

The announcement of Turkey as a European Union (EU) candidate country in the Helsinki Summit (10, 11 December 1999) marked a distinct change of identity policy and attitudes towards its citizens. A result in the shift of mindset has been the launch of the first public service broadcasting TV channel for Kurdish people on the 1st of January 2009. TRT 6 (Şeş) broadcasting in unofficial Kurdish language is run by Turkish Radio and Television Corporation (TRT). The thesis attempts to elaborate on the discussions surrounding the launch of TRT 6, Turkey’s first public service broadcasting TV channel for its Kurdish citizens. The research aims at finding the discourses of multiculturalism and public service broadcasting through the mainstream Turkish newspapers, Cumhuriyet, Hurriyet, Sabah, Taraf and Zaman. The method used for the research is Critical Discourse Analysis (CDA) and the representative newspapers of the Turkish print media are under the question: How has the launch of TRT 6, as the first public service broadcasting channel of Turkey in Kurdish language, been discussed by Turkish daily newspapers in terms of multiculturalism and minority media? The most significant results of the research is that the concerning newspapers have mostly discussed the launch of TRT 6 in the same line with their political affiliation. Thus it is comprehensively concluded that the selected newspapers proved holding a high level of political parallelism, and low professionalism. However, it should be noted that Taraf differs itself from others while challenging the hegemonic discourses embedded in the articles of the other newspapers. Moreover, the study detected three types of discourses: Pro-multiculturalism discourse, Unification discourse, and Assimilation discourse. It can be concluded that in Turkey, media owners and even individual journalists have incentives to form ideological alliances with political parties, and media appears to be an instrument of power struggle. Today, Turkey seems to restore Kurdish identity in its identity policy and aims to proceed with the negotiation for membership of the European Union (EU). The country still strives to transform from the traditional nation-state to a multiethnic democratic state, with multiculturalism as a policy discussed throughout the two terms that the AKP government has been in power. However, this transformation is not an easy process because of the deep-rooted traditions of the nation-state structure that has also polarized the Turkish press.

Counter-figures : An Essay on Antimetaphoric Resistance: Paul Celan's Poetry and Poetics at the Limits of Figurality

Titled "An Essay on Antimetaphoric Resistance", the dissertation investigates what is here being called "Counter-figures": a term which has in this context a certain variety of applications. Any other-than-image or other-than-figure, anything that cannot be exhausted by figuration (and that is, more or less, anything at all, except perhaps the reproducible images and figures themselves) can be considered "counter-figurative" with regard to the formation of images and figures, ideas and schemas, "any graven image, or any likeness of any thing". Singularity and radical alterity, as well as temporality and its peculiar mode of uniqueness are key issues here, and an ethical dimension is implied by, or intertwined with, the aesthetic. In terms borrowed from Paul Celan's "Meridian" speech, poetry may "allow the most idiosyncratic quality of the Other, its time, to participate in the dialogue". This connection between singularity, alterity and temporality is one of the reasons why Celan so strongly objects to the application of the traditional concept of metaphor to poetry. As Celan says, "carrying over [übertragen]" by metaphor may imply an unwillingness to "bear with [mittragen]" and to "endure [ertragen]" the poem. The thesis is divided into two main parts. The first consists of five distinct prolegomena which all address the mentioned variety of applications of the term "counter-figures", and especially the rejection or critique of either metaphor (by Aristotle, for instance) or the concept of metaphor (defined by Aristotle, and sometimes deemed "anti-poetic" by both theorists and poets). Even if we restrict ourselves to the traditional rhetorico-poetical terms, we may see how, for instance, metonymy can be a counter-figure for metaphor, allegory for symbol, and irony for any single trope or for any piece of discourse at all. The limits of figurality may indeed be located at these points of intersection between different types of tropes or figures, and even between figures or tropes and the "non-figurative trope" or "pseudo-figure" called catachresis. The second part, following on from the open-ended prolegomena, concentrates on Paul Celan's poetry and poetics. According to Celan, true poetry is "essentially anti-metaphoric". I argue that inasmuch as we are willing to pay attention to the "will" of the poetic images themselves (the tropes and metaphors in a poem) to be "carried ad absurdum", as Celan invites us to do, we may find alternative ways of reading poetry and approaching its "secret of the encounter", precisely when the traditional rhetorical instruments, and especially the notion of metaphor, become inapplicable or suspicious — and even where they still seem to impose themselves.

War and Revolution in St. Petersburg : Modernist Links in the Poetry of Edith Södergran and Anna Andreevna Akhmatova

Important modernists in their own countries, Anna Akhmatova and Edith Södergran are compared in this dissertation as poets whose poetry reflects the climactic events of the early twentieth century in Finland and Russia. A comparatist, biographical and historical approach is used to uncover the circumstances surrounding these events. First the poets’ early works are reviewed and their contemporaries are mentioned to provide a poetic context. Then a brief review of Finnish and Russian history situates them historically. Next, the rich literary diversity of St. Petersburg’s Silver Age is presented and the work of the poets is viewed in context before their poetry is compared, as the First World War, October Revolution and subsequent Finnish Civil War impact their writing. While biography is not the primary focus, it becomes important as inevitably the writers’ lives are changed by cataclysmic events and the textual analysis of the poems in Swedish, Russian and English shows the impact of war on their poetry. These two poets have not been compared before in a critical review in English and this work contributes to needed work in English. They share certain common modernist traits: attention to the word, an intimate, unconventional voice, and a concern with audience. In addition, they both reject formal traditions while they adopt new forms and use modern, outside influences such as art, architecture and philosophy as subject matter and a lens through which to focus their poetry. While it may seem that Anna Akhmatova was the most socially aware poet, because of the censorship she endured under Stalin, my research has revealed that actually Edith Södergran showed the most social consciousness. Thus, a contrast of the poets’ themes reveals these differences in their approaches. Both poets articulated a vibrant response to war and revolution becoming modernists in the process. In their final works created in the years before their deaths, they reveal the solace they found in nature as well as final mentions of the violent events of their youth. Keywords: St. Petersburg, Modernism, Symbolism, Acmeism, Silver Age, Finland-Swedish literature

Olla elävän sanat : Roolirunon laji Maila Pylkkösen teoksessa Arvo. Vanhaäiti puhuu runonsa

Maila Pylkkönen (1931 1986) was one of the most important modernist poets in Finland and a central figure in developing the dramatic monologue in Finnish literature. The study examines Pylkkönen s poetic work Arvo. Vanhaäiti puhuu runonsa (Value. An old woman speaks her poem, 1959) as an example of the dramatic monologue, approaching it from three perspectives: its generic features and background, and the poetic framework to which it connects in the context of Pylkkönen s poetry. In addition to methods of literary scholarship, the poetic analysis benefits from a linguistic approach. The study shows that the dramatic monologue genre drives Pylkkönen s first work, Klassilliset tunteet (Classical feelings, 1957), in a context of finding poetic identity, characterised by the expression to be the words of a living creature . The study demonstrates that important generic features of the dramatic monologue, namely, a poem representing a speech-event and a hierarchical structure, are also Arvo s most significant generic features. Arvo s poems as speech-events are examined for their internal progressive, pragmatic unity constructed through single line units; for their function as narratives dealing with the life story of an old woman, Arvo s speaker; and from the perspective of the communication between the old woman and the poems other characters. Arvo s speech-events can also be seen as semantic shifts from one poem to another: the poems construct semantic stages representing different phases of the old woman s life. The study demonstrates that analysis of Arvo s hierarchical structure, that is, the relationship between the speaker and the rhetorical levels, reveals the work s structural and ideological wholeness by focusing on the old woman s emotions: longing, loneliness and alienation from the world. In other words, the contradictions between the explicit level of the speaker and an implied rhetorical level open up the tragedy of an old woman s daily life. Study of Arvo s hierarchical structure also highlights the special position of the reader in the framework of a dramatic monologue. The elements of a dramatic present in which the old woman s emotions are conveyed, an italicized opening poem, and the work s title Value invite the reader to consider Arvo as a structural and ideological whole. The function of Arvo s hierarchical structure is to ask the reader to recognise the hopelessness of the old woman s situation, understand it, and even identify with it.

Drag på parnassen. Två sextiotalsstudier. : Del I: Medelklass med mänskligt ansikte. Medelklassradikalism i fyra romaner av Christer Kihlman, Jarl Sjöblom, Marianne Alopaeus och Ulla-Lena Lundberg. Del II: Modernistdebatten. Sak, person och fält i en finlandssvensk litteraturdebatt år 1965.

The objective of my dissertation Pull (or Draught, or Moves) at the Parnassus , is to provide a deeper understanding of Nordic Middle Class radicalism of the 1960 s as featured in Finland-Swedish literature. My approach is cultural materialist in a broad sense; social class is regarded a crucial aspect of the contents and contexts of the novels and literary discussions explored. In the first volume, Middle Class With A Human Face , novels by Christer Kihlman, Jarl Sjöblom, Marianne Alopaeus, and Ulla-Lena Lundberg, respectively, are read from the points of view of place, emotion, and power. The term "cryptotope" is used to designate the hidden places found to play an important role in all of these four narratives. Also, the "chronotope of the provincial small town", described by Mikhail Bakhtin in 1938, is exemplified in Kihlman s satirical novel, as is the chronotope of of war (Algeria, Vietnam) in those of Alopaeus and Lundberg s. All the four novels signal changes in the way general "scripts of emotions", e.g. jealousy, are handled and described. The power relations in the novels are also read, with reference to Michel Foucault. As the protagonists in two of them work as journalists, a critical discussion about media and Bourgeois hegemony is found; the term "repressive legitimation" is created to grasp these patterns of manipulation. The Modernist Debate , part II of the study, concerns a literary discussion between mainly Finland-Swedish authors and critics. Essayist Johannes Salminen (40) provided much of the fuel for the debate in 1963, questioning the relevance to contemporary life of the Finland-Swedish modernist tradition of the 1910 s and 1920 s. In 1965, a group of younger authors and critics, including poet Claes Andersson (28), followed up this critique in a debate taking place mainly in the newspaper Vasabladet. Poets Rabbe Enckell (62), Bo Carpelan (39) and others defended a timeless poetry. This debate is contextualized and the changing literary field is analyzed using concepts provided by sociologist Pierre Bourdieu. In the thesis, the historical moment of Middle Class radicalism with a human face is regarded a temporary luxury that new social groups could afford themselves, as long as they were knocking over the statues and symbols of the Old Bourgeoisie. This is not to say that all components of the Sixties strategy have lost their power. Some of them have survived and even grown, others remain latent in the gene bank of utopias, waiting for new moments of change.

Identification of two novel human neuronal ceroid lipofuscinosis genes

The neuronal ceroid lipofuscinoses (NCLs) are a group of mostly autosomal recessively inherited neurodegenerative disorders. The aim of this thesis was to characterize the molecular genetic bases of these, previously genetically undetermined, NCL forms. Congenital NCL is the most aggressive form of NCLs. Previously, a mutation in the cathepsin D (CTSD) gene was shown to cause congenital NCL in sheep. Based on the close resemblance of the phenotypes between congenital NCLs in sheep and human, CTSD was considered as a potential candidate gene in humans as well. When screened for mutations by sequencing, a homozygous nucleotide duplication creating a premature stop codon was identified in CTSD in one family with congenital NCL. While in vitro the overexpressed truncated mutant protein was stable although inactive, the absence of CTSD staining in brain tissue samples of patients indicated degradation of the mutant CTSD in vivo. A lack of CTSD staining was detected also in another, unrelated family with congenital NCL. These results imply that CTSD deficiency underlies congenital NCL. While initially Turkish vLINCL was considered a distinct genetic entity (CLN7), mutations in the CLN8 gene were later reported to account for the disease in a subset of Turkish patients with vLINCL. To further dissect the genetic basis of the disease, all known NCL genes were screened for homozygosity by haplotype analysis of microsatellite markers and/or sequenced in 13 mainly consanguineous, Turkish vLINCL families. Two novel, family-specific homozygous mutations were identified in the CLN6 gene. In the remaining families, all known NCL loci were excluded. To identify novel gene(s) underlying vLINCL, a genomewide single nucleotide polymorphism scan, homozygosity mapping, and positional candidate gene sequencing were performed in ten of these families. On chromosome 4q28.1-q28.2, a novel major facilitator superfamily domain containing 8 (MFSD8) gene with six family-specific homozygous mutations in vLINCL patients was identified. MFSD8 transcript was shown to be ubiquitously expressed with a complex pattern of alternative splicing. Our results suggest that MFSD8 is a novel lysosomal integral membrane protein which, as a member of the major facilitator superfamily, is predicted to function as a transporter. Identification of MFSD8 emphasizes the genetic heterogeneity of Turkish vLINCL. In families where no MFSD8 mutations were detected, additional NCL-causing genes remain to be identified. The identification of CTSD and MFSD8 increases the number of known human NCL-causing genes to eight, and is an important step towards the complete understanding of the genetic spectrum underlying NCLs. In addition, it is a starting point for dissecting the molecular mechanisms behind the associated NCLs and contributes to the challenging task of understanding the molecular pathology underlying the group of NCL disorders.

Molecular genetic studies on nemaline myopathy and related disorders

Nemaline myopathy (NM) is a rare muscle disorder characterised by muscle weakness and nemaline bodies in striated muscle tissue. Nemaline bodies are derived from sarcomeric Z discs and may be detected by light microscopy. The disease can be divided into six subclasses varying from very severe, in some cases lethal forms to milder forms. NM is usually the consequence of a gene mutation and the mode of inheritance varies between NM subclasses and different families. Mutations in six genes are known to cause NM; nebulin (NEB), alpha-actin, alpha-tropomyosin (TPM3), troponin T1, beta-tropomyosin (TPM2) and cofilin 2, of which nebulin and -actin are the most common. One of the main interests of my research is NEB. Nebulin is a giant muscle protein (600-900 kDa) expressed mainly in the thin filaments of striated muscle. Mutations in NEB are the main cause of autosomal recessive NM. The gene consists of 183 exons. Thus being gigantic, NEB is very challenging to investigate. NEB was screened for mutations using denaturing High Performance Liquid Chromatography (dHPLC) and sequencing. DNA samples from 44 families were included in this study, and we found and published 45 different mutations in them. To date, we have identified 115 mutations in NEB in a total of 96 families. In addition, we determined the occurrence in a world-wide sample cohort of a 2.5 kb deletion containing NEB exon 55 identified in the Ashkenazi Jewish population. In order to find the seventh putative NM gene a genome-wide linkage study was performed in a series of Turkish families. In two of these families, we identified a homozygous mutation disrupting the termination signal of the TPM3 gene, a previously known NM-causing gene. This mutation is likely a founder mutation in the Turkish population. In addition, we described a novel recessively inherited distal myopathy, named distal nebulin myopathy, caused by two different homozygous missense mutations in NEB in six Finnish patients. Both mutations, when combined in compound heterozygous form with a more disruptive mutation, are known to cause NM. This study consisted of molecular genetic mutation analyses, light and electron microscopic studies of muscle biopsies, muscle imaging and clinical examination of patients. In these patients the distribution of muscle weakness was different from NM. Nemaline bodies were not detectable with routine light microscopy, and they were inconspicuous or absent even using electron microscopy. No genetic cause was known to underlie cap myopathy, a congenital myopathy characterised by cap-like structures in the muscle fibres, until we identified a deletion of one codon of the TPM2 gene, in a 30-year-old cap myopathy patient. This mutation does not change the reading frame of the gene, but a deletion of one amino acid does affect the conformation of the protein produced. In summary, this thesis describes a novel distal myopathy caused by mutations in the nebulin gene, several novel nebulin mutations associated with nemaline myopathy, the first molecular genetic cause of cap myopathy, i.e. a mutation in the beta-tropomyosin gene, and a founder mutation in the alpha-tropomyosin gene underlying autosomal recessive nemaline myopathy in the Turkish population.

The Enlightenment Idea of History as a Legitimation Tool of Kemalism in Turkey

The study analyzes the effort to build political legitimacy in the Republic of Turkey by ex-ploring a group of influential texts produced by Kemalist writers. The study explores how the Kemalist regime reproduced certain long-lasting enlightenment meta-narrative in its effort to build political legitimacy. Central in this process was a hegemonic representation of history, namely the interpretation of the Anatolian Resistance Struggle of 1919 1922 as a Turkish Revolution executing the enlightenment in the Turkish nation-state. The method employed in the study is contextualizing narratological analysis. The Kemalist texts are analyzed with a repertoire of concepts originally developed in the theory of narra-tive. By bringing these concepts together with epistemological foundations of historical sciences, the study creates a theoretical frame inside of which it is possible to highlight how initially very controversial historical representations in the end manage to construct long-lasting, emotionally and intellectually convincing bases of national identity for the secular middle classes in Turkey. The two most important explanatory concepts in this sense are di-egesis and implied reader. The diegesis refers to the ability of narrative representation to create an inherently credible story-world that works as the basis of national community. The implied reader refers to the process where a certain hegemonic narrative creates a formula of identification and a position through which any individual real-world reader of a story can step inside the narrative story-world and identify oneself as one of us of the national narra-tive. The study demonstrates that the Kemalist enlightenment meta-narrative created a group of narrative accruals which enabled generations of secular middle classes to internalize Kemalist ideology. In this sense, the narrative in question has not only worked as a tool utilized by the so-called Kemalist state-elite to justify its leadership, but has been internalized by various groups in Turkey, working as their genuine world-view. It is shown in the study that secular-ism must be seen as the core ingredient of these groups national identity. The study proposes that the enlightenment narrative reproduced in the Kemalist ideology had its origin in a simi-lar totalizing cultural narrative created in and for Europe. Currently this enlightenment project is challenged in Turkey by those who are in an attempt to give religion a greater role in Turkish society. The study argues that the enduring practice of legitimizing political power through the enlightenment meta-narrative has not only become a major factor contributing to social polarization in Turkey, but has also, in contradiction to the very real potentials for crit-ical approaches inherent in the Enlightenment tradition, crucially restricted the development of critical and rational modes of thinking in the Republic of Turkey.

Dissection of the genetic background of childhood onset progressive myoclonic epilepsies

The progressive myoclonic epilepsies (PMEs) are a clinically and etiologically heterogeneous group of symptomatic epilepsies characterized by myoclonus, tonic-clonic seizures, psychomotor regression and ataxia. Different disorders have been classified as PMEs. Of these, the group of neuronal ceroid lipofuscinoses (NCLs) comprise an entity that has onset in childhood, being the most common cause of neurodegeneration in children. The primary aim of this thesis was to dissect the molecular genetic background of patients with childhood onset PME by studying candidate genes and attempting to identify novel PME-associated genes. Another specific aim was to study the primary protein properties of the most recently identified member of the NCL-causing proteins, MFSD8. To dissect the genetic background of a cohort of Turkish patients with childhood onset PME, a screen of the NCL-associated genes PPT1, TPP1, CLN3, CLN5, CLN6, MFSD8, CLN8 and CTSD was performed. Altogether 49 novel mutations were identified, which together with 56 mutations found by collaborators raised the total number of known NCL mutations to 364. Fourteen of the novel mutations affect the recently identified MFSD8 gene, which had originally been identified in a subset of mainly Turkish patients as the underlying cause of CLN7 disease. To investigate the distribution of MFSD8 defects, a total of 211 patients of different ethnic origins were evaluated for mutations in the gene. Altogether 45 patients from nine different countries were provided with a CLN7 molecular diagnosis, denoting the wide geographical occurrence of MFSD8 defects. The mutations are private with only one having been established by a founder-effect in the Roma population from the former Czechoslovakia. All mutations identified except one are associated with the typical clinical picture of variant late-infantile NCL. To address the trafficking properties of MFSD8, lysosomal targeting of the protein was confirmed in both neuronal and non-neuronal cells. The major determinant for this lysosomal sorting was identified to be an N-terminal dileucine based signal (9-EQEPLL-14), recognized by heterotetrameric AP-1 adaptor proteins, suggesting that MFSD8 takes the direct trafficking pathway en route to the lysosomes. Expression studies revealed the neurons as the primary cell-type and the hippocampus and cerebellar granular cell layer as the predominant regions in which MFSD8 is expressed. To identify novel genes associated with childhood onset PME, a single nucleotide polymorphism (SNP) genomewide scan was performed in three small families and 18 sporadic patients followed by homozygosity mapping to determine the candidate loci. One of the families and a sporadic patient were positive for mutations in PLA2G6, a gene that had previously been shown to cause infantile neuroaxonal dystrophy. Application of next-generation sequencing of candidate regions in the remaining two families led to identification of a homozygous missense mutation in USP19 for the first and TXNDC6 for the second family. Analysis of the 18 sporadic cases mapped the best candidate interval in a 1.5 Mb region on chromosome 7q21. Screening of the positional candidate KCTD7 revealed six mutations in seven unrelated families. All patients with mutations in KCTD7 were reported to have early onset PME, rapid disease progression leading to dementia and no pathologic hallmarks. The identification of KCTD7 mutations in nine patients and the clinical delineation of their phenotype establish KCTD7 as a gene for early onset PME. The findings presented in this thesis denote MFSD8 and KCTD7 as genes commonly associated with childhood onset symptomatic epilepsy. The disease-associated role of TXNDC6 awaits verification through identification of additional mutations in patients with similar phenotypes. Completion of the genetic spectrum underlying childhood onset PMEs and understanding of the gene products functions will comprise important steps towards understanding the underlying pathogenetic mechanisms, and will possibly shed light on the general processes of neurodegeneration and nervous system regulation, facilitating the diagnosis, classification and possibly treatment of the affected cases.