The neuronal ceroid lipofuscinosis <Emphasis Type="Italic">Cln8</Emphasis> gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy

Background  

The neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative disorders characterized by accumulation of autofluorescent material in many tissues, especially in neurons. Mutations in the CLN8 gene, encoding an endoplasmic reticulum (ER) transmembrane protein of unknown function, underlie NCL phenotypes in humans and mice. The human phenotype is characterized by epilepsy, progressive psychomotor deterioration and visual loss, while motor neuron degeneration (mnd) mice with a Cln8 mutation show progressive motor neuron dysfunction and retinal degeneration.     

On the Gittins index in the M/G/1 queue

For an M/G/1 queue with the objective of minimizing the mean number of jobs in the system, the Gittins index rule is known to be optimal among the set of non-anticipating policies. We develop properties of the Gittins index. For a single-class queue it is known that when the service time distribution is of type Decreasing Hazard Rate (New Better than Used in Expectation), the Foreground–Background (First-Come-First-Served) discipline is optimal. By utilizing the Gittins index approach, we show that in fact, Foreground–Background and First-Come-First-Served are optimal if and only if the service time distribution is of type Decreasing Hazard Rate and New Better than Used in Expectation, respectively. For the multi-class case, where jobs of different classes have different service distributions, we obtain new results that characterize the optimal policy under various assumptions on the service time distributions. We also investigate distributions whose hazard rate and mean residual lifetime are not monotonic.     

A Note on the Integrability of Maximal Functions Related to Differentiation Bases

In this article we study maximal operators related to differentiation bases. In particular, we find a sufficient condition for the integrability of the maximal function. This complements earlier results of Hardy and Littlewood, de Guzmán and Kita. We also give a geometric application of the result.     

Analysis of lipids with desorption atmospheric pressure photoionization-mass spectrometry (DAPPI-MS) and desorption electrospray ionization-mass spectrometry (DESI-MS)

In this article, the effect of spray solvent on the analysis of selected lipids including fatty acids, fat-soluble vitamins, triacylglycerols, steroids, phospholipids, and sphingolipids has been studied by two different ambient mass spectrometry (MS) methods, desorption electrospray ionization-MS (DESI-MS) and desorption atmospheric pressure photoionization-MS (DAPPI-MS). The ionization of the lipids with DESI and DAPPI was strongly dependent on the spray solvent. In most cases, the lipids were detected as protonated or deprotonated molecules; however, other ions were also formed, such as adduct ions (in DESI), [M-H](+) ions (in DESI and DAPPI), radical ions (in DAPPI), and abundant oxidation products (in DESI and DAPPI). DAPPI provided efficient desorption and ionization for neutral and less polar as well as for ionic lipids but caused extensive fragmentation for larger and more labile compounds because of a thermal desorption process. DESI was more suitable for the analysis of the large and labile lipids, but the ionization efficiency for less polar lipids was poor. Both methods were successfully applied to the direct analysis of lipids from pharmaceutical and food products. Although DESI and DAPPI provide efficient analysis of lipids, the multiple and largely unpredictable ionization reactions may set challenges for routine lipid analysis with these methods.     

M/G/1/MLPS compared with M/G/1/PS within service time distribution class IMRL

Multilevel processor-sharing (MLPS) disciplines were originally introduced by Kleinrock (in computer applications 1976) but they were forgotten for years. However, due to an application related to the service differentiation between short and long TCP flows in the Internet, they have recently gained new interest. In this paper we show that, if the service time distribution belongs to class IMRL, the mean delay in the M/G/1 queue is reduced when replacing the PS discipline with any MLPS discipline for which the internal disciplines belong to {FB, PS}. This is a generalization of our earlier result where we restricted ourselves to the service time distribution class DHR, which is a subset of class IMRL.     

Characterisation of organic compounds in aerosol particles from a Finnish forest by on-line coupled supercritical fluid extraction–liquid chromatography–gas chromatography–mass spectrometry

During the European Union project Quantification of Aerosol Nucleation in the European Boundary Layer (QUEST), which began in spring 2003, atmospheric aerosol particles were collected in a Finnish Scots pine forest using a high-volume sampler. The organic compounds in the filter samples were then analysed by on-line coupled supercritical fluid extraction–liquid chromatography–gas chromatography–mass spectrometry (SFE–LC–GC–MS). The sample was first extracted by SFE. During LC the extracts were fractionated into three fractions according to polarity. The final separation was carried out by GC–MS. A fraction volume as high as 840 L was transferred to the GC, using the partial concurrent eluent evaporation technique. The same instrumentation, with an in-situ SFE derivatisation method, was used to analyse organic acids. Major compounds such as n-alkanes and PAH were analysed quantitatively. Their concentrations were lower than those usually observed in urban areas or in other forest areas in Europe. The wind direction was one of the most important factors affecting changes in the daily concentrations of these compounds. Scots pine needles were analysed with the same system to obtain reference data for identification of biogenic compounds in aerosol particles. Other organic compounds found in this study included hopanes, steranes, n-alkanals, n-alkan-2-ones, oxy-PAH, and alkyl-PAH; some biogenic products, including oxidation products of monoterpenes, were also identified.     

Blocking of Dynamic Multicast Connections

Multicast connections have a bandwidth saving nature. This means that a multicast connection – in taking the form of a tree with streams merging at the nodes – requires much less capacity from the network links than a bunch of separate point-to-point connections providing the same connectivity. In this paper, we consider dynamic multicast connections that can be used to model, for example, TV or radio delivery on a telecommunications network, such as an ATM network with virtual circuits. We show how to calculate the blocking probabilities of requests to join such a tree. First, we consider the blocking probabilities occurring in a single link. The resulting model is able to capture heterogeneous capacity requirements for different multicast channels. Then we extend the results to a whole network using the reduced load approximation. The accuracy of the approximation method is studied by simulations.     

Analysis of lipids with desorption atmospheric pressure photoionization‐mass spectrometry (DAPPI‐MS) and desorption electrospray ionization‐mass spectrometry (DESI‐MS)

In this article, the effect of spray solvent on the analysis of selected lipids including fatty acids, fat‐soluble vitamins, triacylglycerols, steroids, phospholipids, and sphingolipids has been studied by two different ambient mass spectrometry (MS) methods, desorption electrospray ionization‐MS (DESI‐MS) and desorption atmospheric pressure photoionization‐MS (DAPPI‐MS). The ionization of the lipids with DESI and DAPPI was strongly dependent on the spray solvent. In most cases, the lipids were detected as protonated or deprotonated molecules; however, other ions were also formed, such as adduct ions (in DESI), [M‐H]⁺ ions (in DESI and DAPPI), radical ions (in DAPPI), and abundant oxidation products (in DESI and DAPPI). DAPPI provided efficient desorption and ionization for neutral and less polar as well as for ionic lipids but caused extensive fragmentation for larger and more labile compounds because of a thermal desorption process. DESI was more suitable for the analysis of the large and labile lipids, but the ionization efficiency for less polar lipids was poor. Both methods were successfully applied to the direct analysis of lipids from pharmaceutical and food products. Although DESI and DAPPI provide efficient analysis of lipids, the multiple and largely unpredictable ionization reactions may set challenges for routine lipid analysis with these methods. Copyright © 2012 John Wiley & Sons, Ltd.