Organized wiring systems

The topics are presented in viewgraph form and include the following: wiring systems, quality control, qualification and standardization, and maintenance costs

NAVAIR aircraft wiring standardization and qualification program

The topics are presented in viewgraph form and include the following: wiring responsibilities; purpose of the program; measurement of program effectiveness; results; and summary

Skatteministeriets kørselsafgifter for lastbiler

Special Session ‐ Skatteministeriets kørselsafgifter for lastbile

De novo mutations in GRIN1 cause extensive bilateral polymicrogyria

Polymicrogyria is a malformation of cortical development. The aetiology of polymicrogyria remains poorly understood. Using whole-exome sequencing we found de novo heterozygous missense GRIN1 mutations in 2 of 57 parent-offspring trios with polymicrogyria. We found nine further de novo missense GRIN1 mutations in additional cortical malformation patients. Shared features in the patients were extensive bilateral polymicrogyria associated with severe developmental delay, postnatal microcephaly, cortical visual impairment and intractable epilepsy. GRIN1 encodes GluN1, the essential subunit of the N-methyl-d-aspartate receptor. The polymicrogyria-associated GRIN1 mutations tended to cluster in the S2 region (part of the ligand-binding domain of GluN1) or the adjacent M3 helix. These regions are rarely mutated in the normal population or in GRIN1 patients without polymicrogyria. Using two-electrode and whole-cell voltage-clamp analysis, we showed that the polymicrogyria-associated GRIN1 mutations significantly alter the in vitro activity of the receptor. Three of the mutations increased agonist potency while one reduced proton inhibition of the receptor. These results are striking because previous GRIN1 mutations have generally caused loss of function, and because N-methyl-d-aspartate receptor agonists have been used for many years to generate animal models of polymicrogyria. Overall, our results expand the phenotypic spectrum associated with GRIN1 mutations and highlight the important role of N-methyl-d-aspartate receptor signalling in the pathogenesis of polymicrogyria

Kinder- und Jugendhilfereport 2018. Eine kennzahlenbasierte Analyse

Der Kinder- und Jugendhilfereport 2018, die zentrale Publikation der Arbeitsstelle Kinder- und Jugendhilfestatistik (AKJStat), beschreibt umfassend die aktuelle Situation und die neuere Entwicklung der Kinder- und Jugendhilfe. Grundlage sind die Daten der amtlichen Kinder- und Jugendhilfestatistik. Erstmals wird die Kinder- und Jugendhilfe auf der Basis von Kennzahlen dargestellt und analysiert. Der Report ermöglicht einen schnellen und zuverlässigen Überblick über zentrale Arbeitsfelder und wichtige Aufgabengebiete. (DIPF/Orig.