Interferon-driven brain phenotype in a mouse model of RNaseT2 deficient leukoencephalopathy

Solid state NMR/Biophysical Organic Chemistr

Potential for more energy efficiency

Bearings provide mechanical movement in countless applications and are an important factor when it comes to energy efficiency. Used materials, lubricants, seals - the factors which Tribology and thus influence energy consumption, depending on the application are quite different to assess. Users will benefit from this background of suppliers such as bearings, in addition to product diversity and the corresponding consulting expertise in terms of energy saving opportunities. Energy savings are being optimized by a bearing design to achieve. Potentials for the improvement of components, for example in material and surface condition of rolling elements and rings, career profile and geometry, the ball or roller set, and its interaction with special cage designs. A lighter course by reduced friction and a lower inertia weight reduced by design - the energy consumption can be reduced in different ways without having to sacrifice in terms of performance and service life must be made. By using the right machine elements can be for example energy-efficient propulsion realize, in addition to cost saving in consumption due to lower demand also reduces CO2 emissions. But what options users have this in detail, they often do not know. Seals and lubricants are other design elements that affect energy efficiency of bearings. They are also in a variety of models available. For example, results from the labyrinth seals a lower power dissipation, they are usually particularly good sealing effect of energy saving. Deep groove ball bearings with low friction seals are not abrasive to the operation of a plant consumption significantly reduced. Compared with a premium solution with grinding of mass of product provides a solution with bearings non abrasive seals not only an efficient operation of the system, but is also a cheaper solution, a further argument for applying the method ABEG in the search for savings potential. The same is true for the lubricants used, which in terms of performance, functionality and durability of the camp plays a key role in the mechanical propulsion role. ABEG Holding Germany advises a holistic and competent around bearings, lubricants and seals. To get users simultaneously powerful and energy efficient storage solutions for the specific purpose

Comparative analysis of alternating hemiplegia of childhood and rapid-onset dystonia-parkinsonism ATP1A3 mutations reveals functional deficits, which do not correlate with disease severity

Heterozygous mutations in the ATP1A3 gene, coding for an alpha subunit isoform (α3) of Na+/K+-ATPase, are the primary genetic cause for rapid-onset dystonia-parkinsonism (RDP) and alternating hemiplegia of childhood (AHC). Recently, cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorineural hearing loss (CAPOS), early infantile epileptic encephalopathy (EIEE), childhood rapid onset ataxia (CROA) and relapsing encephalopathy with rapid onset ataxia (RECA) extend the clinical spectrum of ATP1A3 related disorders. AHC and RDP demonstrate distinct clinical features, with AHC symptoms being generally more severe compared to RDP. Currently, it is largely unknown what determines the disease severity, and whether severity is linked to the degree of functional impairment of the α3 subunit. Here we compared the effect of twelve different RDP and AHC specific mutations on the expression and function of the α3 Na+/K+-ATPase in transfected HEK cells and oocytes. All studied mutations led to functional impairment of the pump, as reflected by lower survival rate and reduced pump current. No difference in the extent of impairment, nor in the expression level, was found between the two phenotypes, suggesting that these measures of pump dysfunction do not exclusively determine the disease severity. GenBank RefSeq accession numbers: ATP1A3: NM_152296.4; ATP1B1: NM_001677.3