Exome sequencing has recently identified mutations in the gene TANGO2 (transport and Golgi organization 2) as a cause of developmental delay associated with recurrent crises involving rhabdomyolysis, cardiac arrhythmias, and metabolic derangements. The disease is not well understood, in part as the cellular function and subcellular localization of the TANGO2 protein remain unknown. Furthermore, the clinical syndrome with its heterogeneity of symptoms, signs, and laboratory findings is still being defined. Here, we describe 11 new cases of TANGO2‐related disease, confirming and further expanding the previously described clinical phenotype. Patients were homozygous or compound heterozygous for previously described exonic deletions or new frameshift, splice site, and missense mutations. All patients showed developmental delay with ataxia, dysarthria, intellectual disability, or signs of spastic diplegia. Of importance, we identify two subjects (aged 12 and 17 years) who have never experienced any overt episode of the catabolism‐induced metabolic crises typical for the disease. Mitochondrial complex II activity was mildly reduced in patients investigated in association with crises but normal in other patients. In one deceased patient, post‐mortem autopsy revealed heterotopic neurons in the cerebral white matter, indicating a possible role for TANGO2 in neuronal migration. Furthermore, we have addressed the subcellular localization of several alternative isoforms of TANGO2, none of which were mitochondrial but instead appeared to have a primarily cytoplasmic localization. Previously described aberrations in Golgi morphology were not observed in cultured skin fibroblasts.
Occupational groups such as firefighters, military officers, paramedics and police officers are exposed to a combination of acute, severe and accumulated everyday stress. Drawing on the daily hassles perspective on stress, the aim was to synthesize existing research on daily hassles in professional first responder settings into a theoretical model. A systematic mixed studies review with an integrated design was undertaken. The selection process resulted in 40 articles meeting the inclusion criteria. The selected papers represented two literature reviews, one qualitative study, eight longitudinal studies and 29 cross-sectional studies. Five superior categories emerged in the analysis: Individual antecedent and continuously framing factors, Environmental antecedent and continuously framing factors, Appraisal and coping processes, Daily hassles and Outcome. Suggestions for future research are presented.
The aim of this study was to investigate whether constructive or destructive leadership behaviors are the best predictors of soldiers' experienced meaningfulness of work and general job satisfaction. Data were collected among 300 employed soldiers using a questionnaire. The questionnaire contained questions about the immediate leader's constructive and destructive leadership, meaningfulness of work and general job satisfaction. The results show that the constructive leadership factor inspiration and motivation was the best predictor of both experienced meaningfulness of work and general job satisfaction. None of the destructive leadership factors gave a significant contribution to the models although both the passive and active forms of destructive leadership showed a weak to moderate significant, negative correlation with experienced meaningfulness of work and general job satisfaction.
The universal nature of conscription ties or anchors broad segments of society to the military. What happens to societal anchoring after the draft ends? Using in-depth interviews with two groups of elites, this article explores factors that promote and hinder the Swedish Military’s societal anchoring post-conscription. The first group consisted of 18 executives at the Swedish Armed Forces (SAF) headquarters with strategic positions in relation to societal anchoring. The second group consisted of 15 representatives from the corresponding political level: members of the parliamentary Committee on Defense and their officials. The interviews were semistructured and analyzed according to a grounded theory-inspired approach. Main themes concerning obstacles were the public’s low interest and dated knowledge, an unclear political debate resulting in vague expectations concerning the SAF, as well as unclear responsibility for informing the public, and confidence gaps between the military and the political elite.
Povzetek Edini način, da na Švedskem postaneš častnik, je, da končaš triletni program šolanja častnikov. V postopku selekcije za program šolanja častnikov je velika razlika med številom kandidatov in številom posameznikov, ki se v resnici začnejo šolati. Ena izmed posledic tega je, da program šolanja častnikov ni bil v celoti zapolnjen že od leta 2008, zaradi česar v švedski vojski primanjkuje častnikov. Prednostna naloga je torej popolnitev švedske vojske z zadostnim številom usposobljenih častnikov, zato se mora od zdaj naprej število kandidatov za častnike povečevati. Leta 2014 se je začel projekt, katerega namen je preučiti pridobivanje kandidatov in selekcijo za program šolanja častnikov ter prepoznavati dejavnike uspešnosti in področja, na katerih so potrebne izboljšave. Dodatna ambicija tega projekta je tudi uvedba ukrepov, potrebnih za povečanje učinkovitosti pridobivanja kandidatov na podlagi rezultatov in razmerja kandidatov, ki se vključijo v program šolanja častnikov. Projekt je privedel do izvajanja drugačne strategije zaposlovanja v letih 2016 in 2017. V začetku postopka selekcije so s prijavljenimi stopili v stik kandidati, ki so že vključeni v program šolanja častnikov, povabljeni pa so bili tudi na informativni sestanek na švedski obrambni univerzi. S tistimi, ki so bili izbrani za šolanje v programu, so nato stopili v stik njihovi prihodnji poveljniki vodov in jim dali koristne informacije. Rezultati kažejo, da se je število kandidatov, ki so se vključili v program šolanja častnikov, povečalo, osip v izbirnem postopku pa je bil manjši kot kadar koli prej. Ključne besede: pridobivanje kadra, častniki, program šolanja častnikov, švedska vojska. Abstract The only way to become an officer in Sweden is to complete the three-year Officers’ Programme (OP). In the selection process for the OP, there is a large gap between the number of applicants and the number of individuals who start the programme. One consequence of this is that the OP has not been fully manned since 2008, thus leaving the Swedish Armed Forces (SAF) with a shortage of officers. Manning the SAF with sufficient officers with the right qualifications is a priority, and henceforth the number of cadets must increase. Therefore, in 2014, a project was launched with the aim of examining recruitment to and selection for the OP, as well as identifying success factors and areas of improvement. An additional ambition was to introduce measures intended to increase recruiting efficiency based on the results, and the proportion of individuals who enter the OP. The project led to the implementation of a different recruitment strategy in 2016 and 2017. Early in the selection process applicants were contacted by cadets already studying on the OP, and were invited to an information meeting at the Swedish Defence University. Those who were selected to start the OP were also contacted by their future platoon commanders, who provided useful information. The outcomes show that the numbers of cadets starting the OP increased, and the dropout rate during the selection process was lower than before. Keywords: Recruitment, Officers, Officers’ Programme, Swedish Armed Forces
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