We report an 8-year-old girl with lower limb weakness since birth in whom mitochondrial trifunctional protein (MTP) deficiency, an autosomal recessive fatty acid oxidation disorder caused by HADHA or HADHB mutations, had not been definitively diagnosed before she was referred to our hospital. Repeated blood acylcarnitine analysis revealed slightly increased long-chain 3-OH-acylcarnitine levels;...

In recent times, RNAi has emerged as an important defence system that regulates replication of pathogens in host cells. Many RNAi related host factors especially the host miRNAs play important roles in all intrinsic cellular functions, including viral infection. We have been working on identification of mammalian host factors involved in Dengue virus infection. In the present study, we identifi...

A limited-area survey of the η Chamaeleontis cluster has identified 2 new late-type members. The more significant of these is ECHA J0843.3–7905 (= IRAS F08450– 7854), a slowly-rotating (P = 12 d) M2 classical T Tauri (CTT) star with a spectrum dominated by Balmer emission. At a distance of 97 pc and cluster age of ≈ 9 Myr, the star is a nearby rare example of an ‘old’ CTT star and promises to b...

Blasticidin A (1) and aflastatin A (2), Streptomyces metabolites with similar structures, are specific inhibitors of aflatoxin production by Aspergillus parasiticus. The stereochemistry of the polyol fragment of 1 (3a) containing ten chiral centers was elucidated by applying acetonide and MTPA methods to a variety of acetonide derivatives of 3a, which determined the absolute configuration of 3a...

The first deadline for REACH registration has passed and registration data for the first set of substances are now public. According to ECHA, 4,599 substances have been registered so far, and the corresponding dossiers of many of them are now partially available in a public database. A sample of 400 records was randomly selected and analyzed with regard to reproductive and developmental toxicit...

Thirteen enantiopure paddlewheel-shaped dirhodium(II) tetrakiscarboxylate complexes have been checked for their efficiency in the dirhodium method (differentiation of enantiomers by NMR spectroscopy); six of them are new. Their diastereomeric dispersion effects were studied and compared via so-called key numbers KN. Adducts of each complex were tested with five different test ligands representi...

Humans do often experience occupational exposure to chemicals, which could lead to negative health effects if the risks aren’t managed. Proper risk assessments of exposure to chemicals is needed and can be performed in different ways. The exposure assessment part of the risk assessment can be performed by exposure measurements or by using exposure assessment models. The use of exposure assessme...

BACKGROUND An Annex XV restriction dossier for cadmium in artists' paints was submitted by an EU member state to the European Chemicals Agency ECHA. By cleaning, used brushes under the tap cadmium can enter the food chain via waste water treatment and subsequent agricultural application of the sewage sludge. It was estimated that 110 kg Cd per year is spread on agricultural land via this exposu...

This paper proposes an extended-flux model with core-loss resistance of SynRMs (synchronous reluctance motors) and precise torque estimation without core-loss measurement and position encoder. The proposed torque estimation is useful for precise MTPA (maximum torque per ampere) control of position sensorless controlled SynRMs, which is achieved with the assistance of active and reactive powers.

Disks in the 6 Myr old cluster η Chamaeleontis were searched for emission from hot H2 . Around the M3 star ECHA J0843.4-7905 we detect circumstellar gas orbiting at ∼2 AU. If the gas is UV-excited, the ro-vibrational line traces a hot gas layer supported by a disk of mass ∼0.03 M⊙ , similar to the minimum mass solar nebula. Such a gas reservoir at 6 Myr would promote the formation and inwards m...