産業用ニッケル水素電池の高出力化とレアメタルフリー化に関する研究

博士（工学）神戸大

Synthesis, photophysics and molecular structures of luminescent 2,5-bis(phenylethynyl)thiophenes (BPETs)

International audienceThe Sonogashira cross-coupling of two equivalents of para-substituted ethynylbenzenes with 2,5-diiodothiophene provides a simple synthetic route for the preparation of 2,5-bis(para-R-phenylethynyl)thiophenes (R = H, Me, OMe, CF3, NMe2, NO2, CN and CO2Me) (1a-h). Likewise, 2,5-bis(pentafluorophenylethynyl)thiophene (2) was prepared by the coupling of 2,5-diiodothiophene with pentafluorophenylacetylene. All compounds were characterised by NMR, IR, Raman and mass spectroscopy, elemental analysis, and their absorption and emission spectra, quantum yields and lifetimes were also measured. The spectroscopic studies of 1a-h and 2 show that both electron donating and electron withdrawing para-subsituents on the phenyl rings shift the absorption and emission maxima to lower energies, but that acceptors are more efficient in this regard. The short singlet lifetimes and modest fluorescence quantum yields (ca. 0.2-0.3) observed are characteristic of rapid intersystem crossing. The single-crystal structures of 2,5-bis(phenylethynyl)thiophene, 2,5-bis(para-carbomethoxyphenylethynyl)thiophene, 2,5-bis(para-methylphenylethynyl)thiophene and 2,5-bis(pentafluorophenylethynyl)thiophene were determined by X-ray diffraction at 120 K. DFT calculations show that the all-planar form of the compounds is the lowest in energy, although rotation of the phenyl groups about the C[triple bond, length as m-dash]C bond is facile and TD-DFT calculations suggest that, similar to 1,4-bis(phenylethynyl)benzene analogues, the absorption spectra in solution arise from a variety of rotational conformations. Frequency calculations confirm the assignments of the compounds' IR and Raman spectra

The clinical and molecular spectrum of galactosemia in patients from the Cape Town region of South Africa

BACKGROUND: The objective of this study was to document the clinical, laboratory and genetic features of galactosemia in patients from the Cape Town metropolitan region. METHODS: Diagnoses were based on thin layer chromatography for galactosuria/galactosemia and assays of erythrocyte galactose-1-phosphate uridyltransferase (GALT) and galactokinase activities. Patients were screened for the common S135L and Q188R transferase gene mutations, using PCR-based assays. Screening for the S135L mutation in black newborns was used to estimate the carrier rate for galactosemia in black South Africans. RESULTS: A positive diagnosis of galactosemia was made in 17 patients between the years 1980 to 2001. All had very low or absent galactose-1-phosphate uridyltransferase (GALT) activity, and normal galactokinase levels. The mean age at diagnosis was 5.1 months (range 4 days to 6.5 months). A review of 9 patients showed that hepatomegaly (9/9), and splenomegaly, failure to thrive, developmental delay, bilateral cataracts (6/9) were the most frequent features at diagnosis. Six had conjugated hyperbilirubinemia. Four experienced invasive E. coli infection before diagnosis. Ten patients were submitted to DNA analysis. All 4 black patients and 2 of mixed extraction were homozygous for the S135L allele, while all 3 white patients were homozygous for the Q188R allele. The remaining patient of mixed extraction was heterozygous for the Q188R allele. The estimated carrier frequency of the S135L mutation in 725 healthy black newborns was 1/60. CONCLUSIONS: In the absence of newborn screening the delay in diagnosis is most often unacceptably long. Also, carrier frequency data predict a galactosemia incidence of approximately 1/14 400 for black newborns in the Cape Metropole, which is much higher than the current detection rate. It is thus likely that many patients go undetected

Dynamic observations of vesiculation reveal the role of silicate crystals in bubble nucleation and growth in andesitic magmas

Bubble nucleation and growth control the explosivity of volcanic eruptions, and the kinetics of these processes are generally determined from examinations of natural samples and quenched experimental run products. These samples, however, only provide a view of the final state, from which the initial conditions of a time-evolving magmatic system are then inferred. The interpretations that follow are inexact due to the inability of determining the exact conditions of nucleation and the potential detachment of bubbles from their nucleation sites, an uncertainty that can obscure their nucleation location \u2013 either homogeneously within the melt or heterogeneously at the interface between crystals and melts. We present results of a series of dynamic, real-time 4D X-ray tomographic microscopy experiments where we observed the development of bubbles in crystal bearing silicate magmas. Experimentally synthesized andesitic glasses with 0.25\u20130.5 wt% H2O and seed silicate crystals were heated at 1 atm to induce bubble nucleation and track bubble growth and movement. In contrast to previous studies on natural and experimentally produced samples, we found that bubbles readily nucleated on plagioclase and clinopyroxene crystals, that their contact angle changes during growth and that they can grow to sizes many times that of the silicate on whose surface they originated. The rapid heterogeneous nucleation of bubbles at low degrees of supersaturation in the presence of silicate crystals demonstrates that silicates can affect when vesiculation ensues, influencing subsequent permeability development and effusive vs. explosive transition in volcanic eruptions

Mucopolysaccharidosis VI

Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B leading to the accumulation of dermatan sulfate. Birth prevalence is between 1 in 43,261 and 1 in 1,505,160 live births. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. The characteristic skeletal dysplasia includes short stature, dysostosis multiplex and degenerative joint disease. Rapidly progressing forms may have onset from birth, elevated urinary glycosaminoglycans (generally >100 μg/mg creatinine), severe dysostosis multiplex, short stature, and death before the 2nd or 3rd decades. A more slowly progressing form has been described as having later onset, mildly elevated glycosaminoglycans (generally <100 μg/mg creatinine), mild dysostosis multiplex, with death in the 4th or 5th decades. Other clinical findings may include cardiac valve disease, reduced pulmonary function, hepatosplenomegaly, sinusitis, otitis media, hearing loss, sleep apnea, corneal clouding, carpal tunnel disease, and inguinal or umbilical hernia. Although intellectual deficit is generally absent in MPS VI, central nervous system findings may include cervical cord compression caused by cervical spinal instability, meningeal thickening and/or bony stenosis, communicating hydrocephalus, optic nerve atrophy and blindness. The disorder is transmitted in an autosomal recessive manner and is caused by mutations in the ARSB gene, located in chromosome 5 (5q13-5q14). Over 130 ARSB mutations have been reported, causing absent or reduced arylsulfatase B (N-acetylgalactosamine 4-sulfatase) activity and interrupted dermatan sulfate and chondroitin sulfate degradation. Diagnosis generally requires evidence of clinical phenotype, arylsulfatase B enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes, and demonstration of a normal activity of a different sulfatase enzyme (to exclude multiple sulfatase deficiency). The finding of elevated urinary dermatan sulfate with the absence of heparan sulfate is supportive. In addition to multiple sulfatase deficiency, the differential diagnosis should also include other forms of MPS (MPS I, II IVA, VII), sialidosis and mucolipidosis. Before enzyme replacement therapy (ERT) with galsulfase (Naglazyme®), clinical management was limited to supportive care and hematopoietic stem cell transplantation. Galsulfase is now widely available and is a specific therapy providing improved endurance with an acceptable safety profile. Prognosis is variable depending on the age of onset, rate of disease progression, age at initiation of ERT and on the quality of the medical care provided

A Study of the Optional Seven Period Day as it is Operating at Logan High School

Education is laboring under a mantle of criticism. This is neither a new nor a unique situation, but if this caviling approach will elevate the quality of education offered the youth of America it should be welcomed by educators and laymen alike