Utsunomiya University

About: Utsunomiya University is a education organization based out in Utsunomiya, Japan. It is known for research contribution in the topics: Laser & Holography. The organization has 4139 authors who have published 6812 publications receiving 91975 citations. The organization is also known as: Utsunomiya daigaku.

Identification of a new complementation group of the peroxisome biogenesis disorders and PEX14 as the mutated gene

Abstract: Peroxisome biogenesis disorders (PBD) are lethal hereditary diseases caused by abnormalities in the biogenesis of peroxisomes. At present, 12 different complementation groups have been identified and to date, all genes responsible for each of these complementation groups have been identified. The peroxisomal membrane protein PEX14 is a key component of the peroxisomal import machinery and may be the initial docking site for the two import receptors PEX5 and PEX7. Although PEX14 mutants have been identified in yeasts and CHO-cells, human PEX14 deficiency has apparently not been documented. We now report the identification of a new complementation group of the peroxisome biogenesis disorders with PEX14 as the defective gene. Indeed, human PEX14 rescues the import of a PTS1-dependent as well as a PTS2-dependent protein into the peroxisomes in fibroblasts from a patient with Zellweger syndrome belonging to the new complementation group. This patient was homozygous for a nonsense mutation in a putative coiled-coil region of PEX14, c.553C>T (p.Q185X). Furthermore, we showed that the patient's fibroblasts lacked PEX14 as determined by immunocytochemical analysis. These findings indicate that there are 13 genotypes in PBD and that the role of PEX14 is also essential in humans. Hum Mutat 23:552-558, 2004. © 2004 Wiley-Liss, Inc.

Observation of thundercloud-related gamma rays and neutrons in Tibet

Abstract: During the 2010 rainy season in Yangbajing (4300 m above sea level) in Tibet, China, a long-duration count enhancement associated with thunderclouds was detected by a solar-neutron telescope and neutron monitors installed at the Yangbajing Comic Ray Observatory. The event, lasting for similar to 40 min, was observed on July 22, 2010. The solar-neutron telescope detected significant gamma-ray signals with energies >40 MeV in the event. Such a prolonged high-energy event has never been observed in association with thunderclouds, clearly suggesting that electron acceleration lasts for 40 min in thunderclouds. In addition, Monte Carlo simulations showed that >10 MeV gamma rays largely contribute to the neutron monitor signals, while >1 keV neutrons produced via a photonuclear reaction contribute relatively less to the signals. This result suggests that enhancements of neutron monitors during thunderstorms are not necessarily clear evidence for neutron production, as previously thought.

A northern sky survey for steady tera-electron volt gamma-ray point sources using the tibet air shower array

Abstract: Results of a steady TeV γ-ray point-source search using data taken from the Tibet HD (1997 February-1999 September) and Tibet III (1999 November-2001 October) arrays are presented. From 0° to 60° in declination, significant excesses from the well-known steady source Crab Nebula and the high state of the flare-type source Markarian 421 are observed. Because the levels of significance from other positions are not sufficiently high, 90% confidence level upper limits on the flux are set assuming different power-law spectra. To allow cross-checking, two independently developed analyses are used in this work.