Neuropathology and Therapeutic Intervention in Spinal and Bulbar Muscular Atrophy

Spinal and bulbar muscular atrophy (SBMA) is a hereditary motor neuron disease caused by the expansion of a polyglutamine tract in the androgen receptor (AR). The histopathological finding in SBMA is loss of lower motor neurons in the anterior horn of the spinal cord as well as in the brainstem motor nuclei. Animal studies have revealed that the pathogenesis of SBMA depends on the level of serum testosterone, and that androgen deprivation mitigates neurodegeneration through inhibition of nuclear accumulation of the pathogenic AR. Heat shock proteins, ubiquitin-proteasome system and transcriptional regulation are also potential targets of therapy development for SBMA

Einfluss der Kambaraerde (saure Bleicherde) auf einige hydrolytische Enzyme

1. Die Kambaraerde verhält sich den Enzymen gegenüber wie eme Säure obwohl ihr Wasserauszug keine Wirkung hat. Diese Eigenschaft kann man bei der technischen Verzuckerung, um das Enzym zu aktivieren, gut verwenden, weil die abfiltrierte Lösung nach der Verzuckerung keine Neutralisation nötig hat, was aber der Fall bei den Mineralsäuren ist. 2. Die optimale Säuremenge wird von den Wirkungsbedingungen bestimmt. Die einfache Mengenangabe ohne Beschreibung der Bedingungen ist sinnlos

Photosynthetic physiology and primary productivity of phytoplankton in the Australian sector of the Southern Ocean

第2回極域科学シンポジウム　共通セッション「海氷圏の生物地球化学」 11月16日（水） 統計数理研究所 3階セミナー

Current Status of Treatment of Spinal and Bulbar Muscular Atrophy

Spinal and bulbar muscular atrophy (SBMA) is the first member identified among polyglutamine diseases characterized by slowly progressive muscle weakness and atrophy of the bulbar, facial, and limb muscles pathologically associated with motor neuron loss in the spinal cord and brainstem. Androgen receptor (AR), a disease-causing protein of SBMA, is a well-characterized ligand-activated transcription factor, and androgen binding induces nuclear translocation, conformational change and recruitment of coregulators for transactivation of AR target genes. Some therapeutic strategies for SBMA are based on these native functions of AR. Since ligand-induced nuclear translocation of mutant AR has been shown to be a critical step in motor neuron degeneration in SBMA, androgen deprivation therapies using leuprorelin and dutasteride have been developed and translated into clinical trials. Although the results of these trials are inconclusive, renewed clinical trials with more sophisticated design might prove the effectiveness of hormonal intervention in the near future. Furthermore, based on the normal function of AR, therapies targeted for conformational changes of AR including amino-terminal (N) and carboxy-terminal (C) (N/C) interaction and transcriptional coregulators might be promising. Other treatments targeted for mitochondrial function, ubiquitin-proteasome system (UPS), and autophagy could be applicable for all types of polyglutamine diseases

Petrology of a new basaltic shergottite: Dhofar 378

Dhofar 378 is a new basaltic shergottite, consisting mainly of pyroxenes, plagioclase glass, phosphates, titanomagnetite, and mesostasis. It is one of the most ferroan shergottites and resembles the Los Angeles shergottite. Pyroxenes show remarkable chemical zoning from 0.4 of Mg/(Mg+Fe) to less than 0.1, and their REE patterns are depleted in light REE whereas the REE pattern of the bulk Dhofar 378 is flat. All plagioclase grains in the original lithology completely melted by an intense impact shock, and the plagioclase melts crystallized fibrous plagioclase to form the rims surrounding the plagioclase melts. Then, the melts quenched as plagioclase glass to form the cores. The shock stage of Dhofar 378 is higher than that of the Los Angeles shergottite. The degree of impact shock for Dhofar 378 may be about 55-75GPa and is the highest among all known martian meteorites