Sounding-rocket experiment to study the diffuse soft X-ray background using a Si(Li) detector

Soft X-ray background in the energy range 0.4 to 10 keV was studied. A payload was developed which uses a wide angle, windowless, cooled, Si(Li) semiconductor detector system. With a resolution of less than 150 eV between 0.3 and 2.0 keV, the system is sensitive to an emission equivalent width of about 10 eV. Carbon and oxygen line emission were detected from the vicinity of the North Galactic Pole and the North Polar Spur

Transcriptional and Post-Transcriptional Regulation of SPAST, the Gene Most Frequently Mutated in Hereditary Spastic Paraplegia

Hereditary spastic paraplegias (HSPs) comprise a group of neurodegenerative disorders that are characterized by progressive spasticity of the lower extremities, due to axonal degeneration in the corticospinal motor tracts. HSPs are genetically heterogeneous and show autosomal dominant inheritance in ∼70–80% of cases, with additional cases being recessive or X-linked. The most common type of HSP is SPG4 with mutations in the SPAST gene, encoding spastin, which occurs in 40% of dominantly inherited cases and in ∼10% of sporadic cases. Both loss-of-function and dominant-negative mutation mechanisms have been described for SPG4, suggesting that precise or stoichiometric levels of spastin are necessary for biological function. Therefore, we hypothesized that regulatory mechanisms controlling expression of SPAST are important determinants of spastin biology, and if altered, could contribute to the development and progression of the disease. To examine the transcriptional and post-transcriptional regulation of SPAST, we used molecular phylogenetic methods to identify conserved sequences for putative transcription factor binding sites and miRNA targeting motifs in the SPAST promoter and 3′-UTR, respectively. By a variety of molecular methods, we demonstrate that SPAST transcription is positively regulated by NRF1 and SOX11. Furthermore, we show that miR-96 and miR-182 negatively regulate SPAST by effects on mRNA stability and protein level. These transcriptional and miRNA regulatory mechanisms provide new functional targets for mutation screening and therapeutic targeting in HSP

Discovery Of An X-ray Qso

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/62636/1/271035a0.pd

INTEGRAL observations of recurrent fast X-ray transient sources

Fast X-ray Transients (FXTs) are believed to be non-recurrent bright X-ray sources lasting less than a day and occurring at serendipitous positions, they can best detected and discovered by instruments having a sufficiently wide field of view and high sensitivity. The IBIS/ISGRI instrument onboard INTEGRAL is particularly suited to detect new or already known fast X-ray transient sources. We report on IBIS/ISGRI detection of newly discovered outbursts of three fast transient sources located at low Galactic latitude: SAX J1818.6-1703; IGR J16479-4514; IGR J17391-302/XTE J1739-302. The reported results confirm and strengthen the very fast transient nature of these sources, given that all their newly detected outbursts have a duration less than about 3 hours. Additionally, they provide the first evidence for a possible recurrent fast transient behaviour as all three sources were detected in outburst by ISGRI more than once during the last 2 years