Pathogenesis of proximal autosomal recessive spinal muscular atrophy

Although it is known that deletions or mutations of the SMN1 gene on chromosome 5 cause decreased levels of the SMN protein in subjects with proximal autosomal recessive spinal muscular atrophy (SMA), the exact sequence of pathological events leading to selective motoneuron cell death is not fully understood yet. In this review, new findings regarding the dual cellular role of the SMN protein (translocation of β-actin to axonal growth cones and snRNP biogenesis/pre-mRNA splicing) were integrated with recent data obtained by detailed neuropathological examination of SMA and control subjects. A presumptive series of 10 pathogenetic events for SMA is proposed as follows: (1) deletions or mutations of the SMN1 gene, (2) increased SMN mRNA decay and reduction in full-length functional SMN protein, (3) impaired motoneuron axonoand dendrogenesis, (4) failure of motoneurons to form synapses with corticospinal fibers from upper motoneurons, (5) abnormal motoneuron migration towards ventral spinal roots, (6) inappropriate persistence of motoneuron apoptosis due to impaired differentiation and motoneuron displacement, (7) substantial numbers of motoneurons continuing to migrate abnormally (“heterotopic motoneurons”) and entering into the ventral roots, (8) attracted glial cells following these heterotopic motoneurons, which form the glial bundles of ventral roots, (9) impaired axonal transport of actin, causing remaining motoneurons to become chromatolytic, and (10) eventual death of all apoptotic, heterotopic and chromatolytic neurons, with apoptosis being more rapid and predominating in the earlier stages, with death of heterotopic and chromatolytic neurons occurring more slowly by necrosis during the later stages of SMA. According to this model, the motoneuron axonopathy is more important for pathogenesis than the ubiquitous nuclear splicing deficit. It is also supposed that individually variable levels of SMN protein, together with influences of other phenotype modifier genes and their products, cause the clinical SMA spectrum through differential degree of motoneuron functional loss

Obesity prevention in younger school-age children

Pretilost ili prekomjerna tjelesna težina problem je modernoga čovjeka. Uzrokovana je neuravnoteženom prehranom, sjedilačkim načinom života te nedostatkom tjelesnoga kretanja. Osim toga, mnogi stručnjaci navode da su uzroci pretilosti: genetsko nasljeđe, uloga majke, loše prehrambene navike roditelja, obogaćivanje prehrane. O kojem god da se uzroku radi neizostavna je činjenica da je prevencija pretilosti preduvjet za normalan rast i razvoj, bilo djeteta, bilo odrasloga čovjeka. Prevencija pretilosti podrazumijeva uravnoteženu i pravilnu prehranu te stvaranje navike redovitoga tjelesnoga kretanja. Vodeći se ovom mišlju o prevenciji pretilosti, odlučila sam provesti istraživanje s učenicima četvrtih razreda kako bih provjerila njihove prehrambene navike kao i naviku redovitoga tjelesnoga kretanja. U istraživanju je sudjelovalo 28 učenika koji su popunili upitnik koji je sadržavao 13 pitanja. Cilj samoga istraživanja je bio potvrditi koliko se teorijski dio, u ovome radu napisan, odnosi na ove učenike kao i kolike su njihove sklonosti prema zdravom načinu življenja koji uključuje uravnoteženu prehranu i redovito tjelesno kretanje. Prva pretpostavka istraživanja koja je glasila ako je hrana koju jedem raznolika, tada će utjecaj na moje zdravlje biti pozitivan je potvrđena jer učenici znaju koje su posljedice neuravnotežene prehrane, a to su potvrdili i svojim odgovorima poput nećemo biti zdravi, imat ćemo višak kilograma, nedostajat će nam vitamina. Pretpostavka ako bavljenje bilo kakvom tjelesnom aktivnošću utječe na moje zdravlje, tada neću imati problema s viškom kilograma je djelomično potvrđena jer učenici znaju vezu između pravilne prehrane i tjelesnoga kretanja te njihova utjecaja na zdravlje, no nigdje nije bilo postavljeno pitanje znaju li ima li to veze s viškom kilograma.Corpulence or fatness is a huge problem of mankind nowadays. It is caused by unbalance diet, too much sitting and lack of movement and exercise. Besides, many experts say that other causes are: genetic reasons, mother's influence, parent's eating habits and food aditions. No matter what caused it, corpulence must be prevented so that each person could have a normal growth. Corpulence prevention includes balanced and healthy diet and regular exercision. That's what brought me to idea of making a survey among fourth graders about their eating habits and exercising. Twenty eight pupils answerd 13 questions. The aim of this survey was a demonstrations of theoretical part about exercising and healthy eating habits how and how much do they practise both in their lives. The first hypothesis, which says if the food I eat is various, the influence to my health will be positive, is confirmed because pupils are aware of consequenses of unbalanced diet. They gave answers like: „We won't be healthy, We'll be overweight, We'll have a lack of vitamins.“ The second hypotesis was if any physical activity affects my health, I won't have problems with my weight. This one is partly confirmed because pupils know how healthy diet and physical activity are connected, but they haven't been asked if it has anything with being overweight

Advantage of four-electrode over two-electrode defibrillators

Defibrillation is the standard clinical treatment used to stop ventricular fibrillation. An electrical device delivers a controlled amount of electrical energy via a pair of electrodes in order to reestablish the normal heart rate. We propose a new technique that is a combination of biphasic shocks applied with a four-electrode system rather than the standard two-electrode system. We use a numerical model of a one-dimensional ring of cardiac tissue in order to test and evaluate the benefit of such a new technique. We compare three different shock protocols, namely, a monophasic and two types of biphasic shocks. The results obtained by using a four-electrode system are compared quantitatively with those obtained with the standard two-electrode system. We find that a huge reduction in defibrillation threshold is achieved with the four-electrode system. For the most efficient protocol (asymmetric biphasic), we obtain a reduction in excess of 80 % in the energy required for a defibrillation success rate of 90 %. The mechanisms of successful defibrillation are also analyzed. This reveals that the advantage of asymmetric biphasic shocks with four electrodes lies in the duration of the cathodal and anodal phase of the shock

Brain asymmetries related to language with emphasis on entorhinal cortex and basal forebrain

Anatomical asymmetries of the human brain are important in at least four respects: 1) they can serve as potential indicators of the evolutionary foundations of language, 2) they can be used for comparative analysis of neural specializations for communication in primates, 3) they may provide underlying structural correlates for functional imaging (fMRI, PET) and genetic studies, and finally 4) they can be used for studying disorders which are suspected to result from either disturbed development of cerebral asymmetry or asymmetric damage to the brain. In the first part of this review, we give a general framework of this field through the brief descriptions of the milestone discoveries and major conceptual advances as they emerged throughout the last 150 years. In the second part, we provide a more detailed view on the functional relevance that asymmetries of the entorhinal cortex and basal forebrain may have on the language

Kajkavizmi u "Tkonskom zborniku"

Predmet ovog rada su kajkavizmi u Tkonskom zborniku – glagoljskom rukopisu koji je početkom 16. stoljeća pisan na frankopanskim posjedima. Utvrđeno je da su u tom rukopisu prisutni kajkavizmi na svim razinama: fonološkoj, morfološkoj, leksičkoj i sintaktičkoj. Najviše je kajkavizama na leksičkoj razini, a oni se mogu podijeliti u dvije skupine: 1. zajednički čakavsko- kajkavski sloj, npr. betegь, gdo, nigdar, hiniti, hud, kaštigati, lotar itd.; 2. kajkavski sloj, npr. fajtati, gorup, nekoteri, pokrivača, škoda, špotati, tanac itd. Prva je kategorija leksema interpolirana u gotovo svim dijelovima CTk, a druga je najčešća u Cvetu od kreposti i Muci. Tkonski zbornik čuva jedno ogromno leksičko bogatstvo, a pri usporedbi pojedinih leksema s onima u hrvatskoglagoljskim misalima i brevijarima, zaključeno je da su neki od njih potvrđeni i ranije, npr. betegь, kaštigati, praviti, gorup, tanac itd. To je potvrda o kontinuitetu hrvatskoglagoljske književnosti. Interpolacija kajkavizama nije ujednačena u svim dijelovima zbornika, kajkavske su intervencije najčešće u Cvetu od kreposti (f. 67 – 85) i u Muci Spasitelja našega (f. 109 – 161). Na temelju provedenog istraživanja može se zaključiti da je Tkonski zbornik rukopis sastavljen iz različitih dijelova, koji nisu nastali u istom razdoblju, ni na istom mjestu. Budući da kajkavizme u pojedinim dijelovima nalazimo na svim razinama (Cvet od kreposti i Muka), može se pretpostaviti da su oni nastali u sjevernom području, tj. bliže kajkavskom.Tkonski zbornik is a glagolitic miscellany with various religious and moral content: ecclesiastical dramas, poems, legends and apocrypha, didactic articles, speeches, Cvet od kreposti, instructions for confessors, blessings, etc. It was written at the beginning of the 16th century somewhere on the property of the Frankopan family. The language of the miscellany is mostly a čakavian-Church Slavonic hybrid with elements of the kajkavian dialect. Infiltration of the kajkavian elements is not equal in all parts of the codex but is most frequent in Cvet od kreposti (67 - 85) and Muka Spasitelja našega (109 - 161). In the Miscellany of Tkon kajkavian elements at the phonological, morphological, lexical and syntactic levels have been attested. The most frequent kajkavian elements are those on the lexical level. They can be divided in two groups: 1. the common čakavian-kajkavian strata, e. g. betegь, gdo, nigdar, hiniti, hud, kaštigati, lotar, etc. 2. kajkavian strata, e. g. fajtati, gorup, nekoteri, pokrivača, škoda, špotati, tanac, etc. The lexemes of the first group are infiltrated in almost all of the parts of the Miscellany. The lexemes of the second group are the most frequent in Cvet od kreposti and Muka. The comparison of certain lexemes with those of croato-glagolitic missals and breviaries has shown that some of them were attested earlier, e. g. betegь, kaštigati, praviti, gorup, tanac, etc. It confirms the continuity of croato-glagolitic literacy. The conclusion is that the Miscellany of Tkon is a manuscript combined from various parts which originated neither in the same time nor in the same location. As kajkavian elements are found in all levels of the language we can assume that Cvet od kreposti and Muka originated in the northern area i. e. somewhat closer to the kajkavian area

Neuroticism in the function of the menstrual cycle phases

Cilj provedenog istraživanja bio je ispitati neuroticizam u funkciji različitih faza menstrualnog ciklusa. U ispitivanju longitudinalnog tipa sudjelovale su zdrave ispitanice s regularnim menstrualnim ciklusima. Za određivanje neuroticizma korištena je skala neuroticizma Eysenckovog upitnika ličnosti. Ispitanice su popunjavanje skalu u različitim fazama menstrualnog ciklusa (menstrualna, folikularna, ovulatorna, lutealna i predmenstrualna faza). Izraženiji neuroticizam u menstrualnoj i predmenstrualnoj fazi, u skladu je s očekivanjima, imajući u vidu sniženu razinu spolnih hormona u organizmu, te prisustvo somatskih simptoma i negativnih raspoloženja u navedenim fazama ciklusa. U ostatku ciklusa, tj. u folikularnoj, ovulatornoj i lutealnoj fazi evidentno je smanjenje neuroticizma.The aim of this investigation was to examine neuroticism in the function of different menstrual cycle phases. Healthy female subjects with a regular menstrual cycle took part in this longitudinal study. In order to determine the neuroticism level, the neuroticism scale of the Eysenck Personality Inventory was used. This scale was filled during different phases of the menstrual cycle (menstrual, follicular, ovulatory, luteal and premenstrual phase). A higher level of neuroticism during the premenstrual and menstrual phase of the cycle is to be expected, considering a reduction of sex hormones in the body, presence of somatic symptoms and negative moods in these phases of the cycle. A decrease in neuroticism is evident during the rest of the menstrual cycle, ie. during the follicular, ovulatory and luteal phases of the cycle

Analiza helikopterskog rotora u vertikalnom letu primjenom teorije noseće linije

Tema ovog završnog rada je teorija noseće linije i njena implementacija na model helikopterskog rotora primjenjenog u vertikalnom letu. U skladu s tim razvijen je programski alat za modeliranje jednostavnog diskretiziranog osnosimetričnog rotora za potrebe analize prema navedenoj teoriji. Analiza je provedena uzimajući u obzir samo rotor kao sastavni dio letjelice, pretpostavljajući neometano osnosimetrično strujanje i sukladno s time očekivanu geometriju traga rotora. Za zglob mahanja pretpostavljeno je da se nalazi u osi simetrije rotora, a zglob zabacivanja je zanemaren. \Numerička implementacija modela izvedena je u MATLAB-u, a dobiveni rezultati uspoređeni su s dostupnim eksprerimentalnim podacima i rezultatima prema teoriji elementarnog kraka, a primjenjenoj na konkretnim rotorima. Prilikom proračuna pretpostavljeni su maleni iznosi Machovog broja, tako da nisu uzeti u obzir utjecaj stlačivosti kao niti gubici na vrhovima krakova. Analiza je obuhvatila promjenu inteziteta vrtloga po rasponu kraka rotora, preko koje su se tražili ukupni koeficijenti pogonske sile i snage, a u cilju što boljeg pojašnjenja prikazana je i većina međurezultata

Areas of Implementation of Ethnic Rights of Members of National Minorities in the Republic of Croatia

The author gives an outline of constitutional and legal provisions regulating the rights of ethnic minorities in the Republic of Croatia as well as the site-based policy of the protection of minority rights. The major areas in which the Government of the Republic of Croatia has been supporting the activities of the minority groups’ organisations are: publishing, cultural societies, libraries, minority curricula, preservation of the minority cultural heritage and research projects. Between 1992 and 1997, the Government of the Republic of Croatia earmarked 22 million DEM for the minorities’ activities. The author concludes that the ethnic minorities in Croatia, despite the political and economic hardships, have enjoyed a high degree of minority rights and freedoms

On the Newer Literary-Critical Approach to Biblical Poetry

This presentation discusses the views of three renowned authors – James Kugel, Robert Alter, and Jan Fokkelman – known for their literary-critical approach to the Bible, concerning biblical poetry. According to Kugel, looking at the Bible through the lens of division into poetry and prose (lyrical and epic literature) means looking at it wrongly. He maintains that even meticulous analyses of parallelism can be distorted if viewed through this lens. Therefore, Kugel asserts that there is no poetry in the Bible but rather a “continuum” of loosely connected parallel structures in what we see as prose sections and “heightened rhetoric” in what we often erroneously consider verses. According to Alter, biblical poetry is based on semantic parallelism. However, he points out that poetic expression deliberately avoids complete parallelism, just as language resists mere synonyms by introducing subtle differences between related terms. In contrast, Fokkelman believes that combining prose and poetry, and even transitioning between them, is possible because most Hebrew sentences contain two to eight words and are usually linked in sequences through parataxis (using “… and… and… but… and then”). All three opinions lead to the conclusion that biblical poetry, like prose, is to a large extent sui generis, and that any distinction between poetry and prose, if it exists at all, is not of the same nature as in Western literary culture and it is, therefore, inappropriate to refer to prosimetrum in the Bible

Areas of Implementation of Ethnic Rights of Members of National Minorities in the Republic of Croatia

The author gives an outline of constitutional and legal provisions regulating the rights of ethnic minorities in the Republic of Croatia as well as the site-based policy of the protection of minority rights. The major areas in which the Government of the Republic of Croatia has been supporting the activities of the minority groups’ organisations are: publishing, cultural societies, libraries, minority curricula, preservation of the minority cultural heritage and research projects. Between 1992 and 1997, the Government of the Republic of Croatia earmarked 22 million DEM for the minorities’ activities. The author concludes that the ethnic minorities in Croatia, despite the political and economic hardships, have enjoyed a high degree of minority rights and freedoms