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There is a growing gap between higher education systems and the needs and demands of the labor market. Many of today’s students will work at jobs that just have emerged or do not even exist yet. The “newcomers” will need both digital and social-emotional skills in the coming years. These new competencies will make the new generation of employees’ company goals. This article presents the results of the recent research about modern-day competencies to evaluate what exactly relevant companies’ expectations are, how students see their knowledge and value in future workplaces, and how academia is coping with this new demand. For this analysis, I have conducted deep interviews with applicable entities, namely companies from the car industry and from the field of security industries (cyber security, integrated camera surveillance, financial security) to see how Industry 4.0 shapes the competencies they expect from our students entering to the job market. Engineering students - by questionnaire -were also interviewed at the Óbuda University, to examine their views about the skills gained at the university and how these competencies helping them to apply for the right position in the job market. Although the competence list showed similarities in the expected skill sets, the order of them differs. While most companies are aiming to hire team players with creative problem solving and those are capable and willing to accept changes, the students’ observations showed that technical skills, expertise, and problem solving are the most important competencies for future employment. Based on all participants’ answers and additional research, we aim to involve international companies to take part in our higher education system more thoroughly either by widening the practical in-site education or by inviting them to our university for lecturing future engineers. Furthermore, new courses are introduced at our university, such as information security, humanitarian response management, rehabilitation environmental planning engineering or ergonomics and human factors specialization.

beta-Ureidopropionase deficiency is an inborn error of the pyrimidine degradation pathway, affecting the cleavage of N-carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid. In this study, we report the elucidation of the genetic basis underlying a beta-ureidopropionase deficiency in four patients presenting with neurological abnormalities and strongly elevated levels of N-carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid in plasma, cerebrospinal fluid and urine. No beta-ureidopropionase activity could be detected in a liver biopsy obtained from one of the patients, which reflected the complete absence of the beta-ureidopropionase protein. Analysis of the beta-ureidopropionase gene (UPB1) of these patients revealed the presence of two splice-site mutations (IVS1-2A>G and IVS8-1G>A) and one missense mutation (A85E). Heterologous expression of the mutant enzyme in Escherichia coli showed that the A85E mutation resulted in a mutant beta-ureidopropionase enzyme without residual activity. Our results demonstrate that the N-carbamyl-beta-amino aciduria in these patients is due to a deficiency of beta-ureidopropionase, which is caused by mutations in the UPB1 gene. Furthermore, an altered homeostasis of beta-aminoisobutyric acid and/or increased oxidative stress might contribute to some of the clinical abnormalities encountered in patients with a beta-ureidopropionase deficiency. An analysis of the presence of the two splice site mutations and the missense mutation in 95 controls identified one individual who proved to be heterozygous for the IVS8-1G>A mutation. Thus, a beta-ureidopropionase deficiency might not be as rare as is generally considered.