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Molybdenum cofactor deficiency is an autosomal recessive inborn error of metabolism, which results from mutations in genes involved in Moco biosynthesis. Moco serves as a cofactor of several enzymes, including sulfite oxidase. MoCD is clinically characterized by intractable seizures and severe, rapidly progressing neurodegeneration leading to death in early childhood in the majority of known cases. Here we report a patient with an unusual late disease onset and mild phenotype, characterized by a lack of seizures, normal early development, a decline triggered by febrile illness and a subsequent dystonic movement disorder. Genetic analysis revealed a homozygous c.1338delG MOCS1 mutation causing a frameshift (p.S442fs) with a premature termination of the MOCS1AB translation product at position 477 lacking the entire MOCS1B domain. Surprisingly, urine analysis detected trace amounts (1% of control) of the Moco degradation product urothione, suggesting a residual Moco synthesis in the patient, which was consistent with the mild clinical presentation. Therefore, we performed bioinformatic analysis of the patient’s mutated MOCS1 transcript and found a potential Kozak-sequence downstream of the mutation site providing the possibility of an independent expression of a MOCS1B protein. Following the expression of the patient’s MOCS1 cDNA in HEK293 cells we detected two proteins: a truncated MOCS1AB protein and a 22.4 kDa protein representing MOCS1B. Functional studies of both proteins confirmed activity of MOCS1B, but not of the truncated MOCS1AB. This finding demonstrates an unusual mechanism of translation re-initiation in the MOCS1 transcript, which results in trace amounts of functional MOCS1B protein being sufficient to partially protect the patient from the most severe symptoms of MoCD.
In the context of the Franco-German research project Re(h)strain, this work focuses on a global system analysis integrating both safety and security analysis of international and/or urban railway stations. The Re(h)strain project focuses on terrorist attacks on high speed train systems and investigates prevention and mitigation measures to reduce the overall vulnerability and strengthen the system resilience. One main criterion regarding public transport issues is the number of passengers. For example, the railway station of Paris “Gare du Nord” deals with a bigger number of passengers than the biggest airport in the world (SNCF open Data 2014), the Atlanta airport, but in terms of passengers, it is only around the 23rd rank railway station in the world. Due to the enormous mass of people, this leads to the system approach of breaking out the station into several classes of zones, e.g. entrance, main hall, quays, trains, etc. All classes are analysed considering state-of-the-art parameters, like targets attractiveness, feasibility of attack, possible damage, possible mitigation and defences. Then, safety incidence of security defence is discussed in order to refine security requirement with regard to the considered zone. Finally, global requirements of security defence correlated to the corresponding class of zones are proposed.
Pozzolanic properties of Pennisetum purpureum grass ash were tested on Portland cement. Results show that the ash can be blended with cements without compromising binding strength of the cement. It was found that Portland cement could be blended with Pennisetum purpureum up to a ratio of 3:2 compromising compressive strength of mortar.Mortar with lower cement replacement took longer to set as evidenced by lower compressive strength within the 28-day aging time. Mortar with higher cement replacement had lower water absorption capacity, an indication that the test pozzolan was of smaller particulate size. XRF analysis and the FTIR spectrum showed that the ash has a higher content of silica. The XRD pattern of the ash showed that the ash was predominantly amorphous. SEM images showed that the ash produced at 600 o C had residual carbon material.
Als rohstoffarme und exportorientierte Wirtschaftsnation ist die Bundesrepublik in ho- hem Maß auf die Sicherung und Sicherheit der Logistikketten im grenzüberschreiten- den Verkehr angewiesen. Angesichts der komplexen Transportstrukturen bei grenz- überschreitenden Transporten kommt den eingesetzten Kontroll- und Prüfverfahren besondere Bedeutung zu: Einerseits müssen Kostenbelastungen, Unterbrechungen und Verzögerungen in der Transportkette minimiert, andererseits besonders illegale Einfuhren, Transporte und Substanzen unterbunden werden. Von besonderer Bedeu- tung für Verdachts- bzw. Stichprobenkontrollen ist der Einsatz speziell trainierter Spür- hunde. Als besonders leistungsfähige ‚lebende Sensoren‘ sind sie in der Lage, eine Vielzahl von Stoffen zu detektieren. Der Einsatz von Spürhunden unterliegt allerdings engen Grenzen: Hoher Trainingsaufwand, eng begrenzte Einsatzdauer, begrenzte Verfügbarkeit. Die Entwicklung neuer, optimierter Einsatzverfahren für Spürhunde z. B. mit höheren Durchsatzraten und überprüfbarer Verlässlichkeit durch Einbindung technischer Systeme ist daher ein wichtiger Beitrag für die Sicherung und Sicherheit der Logistikketten.
Amino acids perform multiple essential physiological roles in humans, and accordingly, their importance to health has been the subject of extensive attention. In this special issue of the Journal of Nutrition and Metabolism, we focus on the various inborn errors of amino acid metabolism, their diagnostic challenges, new treatment approaches, and recent advances in patient monitoring as well as clinical outcomes.
After replanting apple (Malus domestica Borkh.) on the same site severe growth suppressions, and a decline in yield and fruit quality are observed in all apple producing areas worldwide. The causes of this complex phenomenon, called apple replant disease (ARD), are only poorly understood up to now which is in part due to inconsistencies in terms and methodologies. Therefore we suggest the following definition for ARD: ARD describes a harmfully disturbed physiological and morphological reaction of apple plants to soils that faced alterations in their (micro-) biome due to the previous apple cultures. The underlying interactions likely have multiple causes that extend beyond common analytical tools in microbial ecology. They are influenced by soil properties, faunal vectors, and trophic cascades, with genotype-specific effects on plant secondary metabolism, particularly phytoalexin biosynthesis. Yet, emerging tools allow to unravel the soil and rhizosphere (micro-) biome, to characterize alterations of habitat quality, and to decipher the plant reactions. Thereby, deep insights into the reactions taking place at the root rhizosphere interface will be gained. Counteractions are suggested, taking into account that culture management should emphasize on improving soil microbial and faunal diversity as well as habitat quality rather than focus on soil disinfection.
Here, we present a miR mechanism which is active in the nucleus and is essential for the production of intron included, C-terminal truncated and biologically active proteins, like e.g. Vim3. We exemplified this mechanism by miRs, miR-15a and miR-498, which are overexpressed in clear cell renal carcinoma or oncocytoma. Both miRs directly interact with DNA in an intronic region, leading to transcriptional stop, and therefore repress the full length version of the pre-mRNA, resulting in intron included truncated proteins (Mxi-2 and Vim3). A computational survey shows that this miR:DNA interactions mechanism may be generally involved in regulating the human transcriptome, with putative interaction sites in intronic regions for over 1000 genes. In this work, an entirely new mechanism is revealed how miRs can repress full length protein translation, resulting in C-terminal truncated proteins.
