Digital measures of respiratory and upper limb function in spinal muscular atrophy: design, feasibility, reliability, and preliminary validity of a smartphone sensor-based assessment suite.

Spinal muscular atrophy (SMA) is characterized by progressive muscle weakness and paralysis. Motor function is monitored in the clinical setting using assessments including the 32-item Motor Function Measure (MFM-32), but changes in disease severity between clinical visits may be missed. Digital health technologies may assist evaluation of disease severity by bridging gaps between clinical visits.

Moderation of Structural DNA Properties by Coupled Dinucleotide Contents in Eukaryotes.

Dinucleotides are known as determinants for various structural and physiochemical properties of DNA and for binding affinities of proteins to DNA. These properties (e.g.

Chemical synthesis and characterization of single-chain C-chloroparaffin materials with defined degrees of chlorination.

Technical chlorinated paraffins (CPs) are produced via radical chlorination of n-alkane feedstocks with different carbon chain-lengths (∼C-C). Short-chain CPs (SCCPs, C-C) are classified as persistent organic pollutants (POPs) under the Stockholm Convention. This regulation has induced a shift to use longer-chain CPs as substitutes.

A new synthesis approach for the generation of single chain CP mixtures composed of a few major compounds.

Chlorinated paraffins (CPs) are complex mixtures, which consist of thousands of individual compounds with no dominant representative. Consequently, knowledge on structure and environmental relevance of individual CP congeners is poor. Similarly to the synthesis of individual CPs, the generation of less complex CP mixtures that can be thoroughly analyzed may be used to overcome some drawbacks of the highly complex technical CP mixtures.

Chlorinated paraffins - A historical consideration including remarks on their complexity.

Chlorinated paraffins (CPs) are high production volume chemicals currently produced and used in higher quantities than any other medium-size polyhalogenated compound (class). In addition, the composition of industrial CP mixtures is highly complex and poorly understood. In this article, we searched in the literature for the beginning of the chlorination of alkanes and how this substance class developed from niche applications to unmatched quantities in various industrial applications.

Determination of chlorinated paraffins (CPs): Analytical conundrums and the pressing need for reliable and relevant standards.

The determination of chlorinated paraffins (CPs) has posed an intractable challenge in analytical chemistry for over three decades. The combination of an as yet unspecifiable number (tens - hundreds of thousands) of individual congeners in mass produced commercial CP mixtures and the steric interactions between them, contrive to defy efforts to characterise their residual occurrences in environmental compartments, food and human tissues. However, recent advances in instrumentation (mass spectrometric detectors and nuclear magnetic resonance), combined with interlaboratory studies, have allowed a better insight into the nature of the conundrums.

Transport of chlorinated paraffins (CPs) from baking oven doors into the food.

Chlorinated paraffins (CPs) have been repeatedly detected in the kitchen environment. Especially baking ovens were contaminated with high CP amounts on the insides of the doors. To investigate if CPs could be transferred into baked food, we spiked self-synthesized single chain C-CP and C-CP standards onto the inside door of an unused, CP-free baking oven.

Chlorinated paraffins in hinges of kitchen appliances.

Chlorinated paraffins (CPs) are anthropogenic pollutants of growing environmental concern. These highly complex mixtures of thousands of homologs and congeners are usually applied as additives in lubricants or as flame retardants and plasticizers in polymers and paints. Recent studies indicated the presence of high amounts of CPs in the kitchen environment whose sources could not be unequivocally identified.

In vitro human cell-based TTR-TRβ CALUX assay indicates thyroid hormone transport disruption of short-chain, medium-chain, and long-chain chlorinated paraffins.

Over the last decades, short-chain chlorinated paraffins (SCCPs), medium-chain chlorinated paraffins (MCCPs), and long-chain chlorinated paraffins (LCCPs) have become the most heavily produced monomeric organohalogen compound class of environmental concern. However, knowledge about their toxicology is still scarce, although SCCPs were shown to have effects on the thyroid hormone system. The lack of data in the case of MCCPs and LCCPs and the structural similarity with perfluoroalkyl substances (PFAS) prompted us to test CPs in the novel TTR-TR CALUX assay for their thyroid hormone transport disrupting potential.

NMR and GC/MS analysis of industrial chloroparaffin mixtures.

Chlorinated paraffins (CPs) are high-volume chemicals used worldwide in various industries as plasticizers, lubricants, and flame retardants. CPs are produced by chlorination of alkane mixtures which leads to complex products of thousands of homologs and congeners. Classic mass spectrometric analyses of CPs allow determining carbon chain lengths and degrees of chlorination while information on the substitution patterns cannot be derived.

Characterization of synthetic single-chain CP standard materials - Removal of interfering side products.

The photolytic chlorination of n-alkanes in presence of sulfuryl chloride (SOCl) was explored to produce new standard materials. Five mixtures of chlorinated tetradecanes were synthesized with chlorination degrees (m) varying from 43.7% to 59.

High amounts of chlorinated paraffins in oil-based vitamin E dietary supplements on the German market.

Chlorinated paraffins (CPs) are a group of man-made pollutants of growing environmental concern. Short-chain chlorinated paraffins (SCCPs) were recently classified as persistent organic pollutants (POPs), while medium-chain chlorinated paraffins (MCCPs) are still unregulated. Foodstuff is a major pathway for the human CP intake, and the regular diet has been analyzed in several studies recently.

Characterization of single chain length chlorinated paraffin mixtures with nuclear magnetic resonance spectroscopy (NMR).

Chlorinated paraffins (CPs) are complex mixtures of polychlorinated n-alkanes of different chain length. Despite their environmental relevance, quantification is still a challenge. Moreover, the individual structures of the molecules in technical CP mixtures are largely unknown.

Photolytic Transformation Products of Decabromodiphenyl Ethane (DBDPE).

The photolytic transformation of decabromodiphenyl ethane-a current-use brominated flame retardant and major substitute of the structurally related decabromodiphenyl ether-was investigated in different solvents (toluene, dichloromethane, chlorobenzene, and benzyl alcohol). The transformation rate followed pseudo first order kinetics, with increasing half-life ( t) in the order of toluene ( t = 4.6 min), chlorobenzene ( t = 14.

Synthesis and characterization of eight single chain length chlorinated paraffin standards and their use for quantification.

Rationale: Quantification of chlorinated paraffins (CPs) via gas chromatography coupled with electron capture negative ionization low-resolution mass spectrometry (GC/ECNI-MS) is a very common practice, although the quality of the results has been disputed due to insufficient mass resolution and the strong impact of the degree of chlorination on the GC/ECNI-MS response.

Methods: For further tests we synthesized eight single chain length CP standards (C -C ) with chlorine contents between 51.1% and 61.

High levels of medium-chain chlorinated paraffins and polybrominated diphenyl ethers on the inside of several household baking oven doors.

Fat obtained by wipe tests on the inner surface of 21 baking ovens from Stuttgart (Germany) were analyzed for halogenated flame retardants (HFRs), namely polybrominated diphenyl ethers (PBDEs), decabromodiphenyl ethane (DBDPE), dechlorane plus (DP), short- and medium-chain chlorinated paraffins (SCCPs, MCCPs), as well as polychlorinated biphenyls (PCBs). In ~50% of the samples chlorinated paraffins (CPs) were present in the mg/g fat range, i.e.

Industrial methodology for process verification in research (IMPROVER): toward systems biology verification.

Motivation: Analyses and algorithmic predictions based on high-throughput data are essential for the success of systems biology in academic and industrial settings. Organizations, such as companies and academic consortia, conduct large multi-year scientific studies that entail the collection and analysis of thousands of individual experiments, often over many physical sites and with internal and outsourced components. To extract maximum value, the interested parties need to verify the accuracy and reproducibility of data and methods before the initiation of such large multi-year studies.

Information systems for surrogate biomarkers.

Biopharmaceutical industry is incorporating biomarker concepts throughout the R&D processes, including the inclusion of biomarker-related data in the submission of new drug applications. In this paper we discuss the role of IT and information systems that support incorporation of surrogate biomarkers in the clinical development process, with emphasis on new molecular imaging technologies and associated IT requirements. In addition, we discuss how recently introduced FDA standards regarding submission data and FDA guidance documents related to genomic and imaging data can be accommodated in a solution architecture for (surrogate) biomarker-based clinical development.

Characterization of platelet-derived growth factor-C (PDGF-C): expression in normal and tumor cells, biological activity and chromosomal localization.

The predicted platelet-derived growth factor-C (PDGF-C) polypeptide contains an N-terminal CUB-like domain and a C-terminal domain with homology to members of the PDGF/vascular endothelial growth factor (VEGF) family. PDGF-C mRNA is widely expressed in normal tissues and does not appear to be up-regulated in the tumor cell lines tested. The PDGF-C gene was mapped to human chromosome 4q31-32.

Cloning and analysis of a novel human putative DNA methyltransferase.

DNA methylation is intricately involved in a variety of cellular processes, such as differentiation, cell cycle progression, X-chromosome inactivation and genomic imprinting. However, little is known about how specific DNA methylation patterns are established and maintained. Previously one mammalian DNA methyltransferase has been described, but there has been considerable speculation about the presence of a second activity capable of methylation.

Nucleotide sequence of human adenovirus type 12 DNA: comparative functional analysis.

A fresh inoculum of human adenovirus type 12 (Ad12) was obtained from the American Type Culture Collection and passaged once on human embryonic kidney cells, and Ad12 DNA was prepared from the first-passage yield to avoid higher passages which might have generated host-virus DNA recombinants. The 18 PstI fragments of Ad12 DNA were cloned into the pBluescript KS vector, and the entire nucleotide sequence of both strands from all 18 fragments was determined by using successive oligodeoxyribonucleotide primers. Ad12 DNA extends over 34,125 nucleotide pairs, and its molecular weight is calculated to be about 22 x 10(6).

Patterns of DNA methylation in selected human genes in different Hodgkin's lymphoma and leukemia cell lines and in normal human lymphocytes.

The human genome, like many other genomes, harbors highly specific patterns of DNA methylation which have not yet been systematically studied. In a limited investigation on the genes for tumor necrosis factors-alpha and -beta, a surprising interindividual concordance in the patterns of DNA methylation at the nucleotide level has been demonstrated earlier by using the genomic sequencing method on DNA from individuals of very different ethnic origins. Patterns of DNA methylation could perhaps serve as indicators for genetic activities.