Mammalian Models of Duchenne Muscular Dystrophy: Pathological Characteristics and Therapeutic Applications

Duchenne muscular dystrophy (DMD) is a devastating X-linked muscle disorder characterized by muscle wasting which is caused by mutations in the DMD gene. The DMD gene encodes the sarcolemmal protein dystrophin, and loss of dystrophin causes muscle degeneration and necrosis. Thus far, therapies for this disorder are unavailable. However, various therapeutic trials based on gene therapy, exon skipping, cell therapy, read through therapy, or pharmaceutical agents have been conducted extensively. In the development of therapy as well as elucidation of pathogenesis in DMD, appropriate animal models are needed. Various animal models of DMD have been identified, and mammalian (murine, canine, and feline) models are indispensable for the examination of the mechanisms of pathogenesis and the development of therapies. Here, we review the pathological features of DMD and therapeutic applications, especially of exon skipping using antisense oligonucleotides and gene therapies using viral vectors in murine and canine models of DMD

Game Production Studies

Video games have entered the cultural mainstream and in terms of economic profits they now rival established entertainment industries such as film or television. As careers in video game development become more common, so do the stories about precarious working conditions and structural inequalities within the industry. Yet, scholars have largely overlooked video game production cultures in favor of studying games themselves and player audiences. In Game Production Studies, an international group of established and emerging researchers takes a closer look at the everyday realities of video game production, ranging from commercial industries to independent creators and cultural intermediaries. Across sixteen chapters, the authors deal with issues related to labour, game development, monetization and publishing, as well as local specificities. As the first edited collection dedicated solely to video game production, this volume provides a timely resource for anyone interested in how games are made and at what costs

Establishment of Nonlife Insurance Business Navigation System A-NIBNS

In this study, the authors teamed up with nonlife insurance companies and agencies to create a business approach method that made it possible to reform insurance-related business activities from a policyholder perspective. A detailed operational method was then put together as A-NIPNS, or Amalabs Nonlife Insurance Business Navigation System. The system is comprised of four parts: (1) a networking system for sharing information within nonlife insurance companies and with agencies, (2) a customer handling evaluation system, (3) a business information sharing system used to link nonlife insurance companies and agencies, and (4) a complaint utilization system for policyholders. After putting together the system, the authors implemented a trial operation using Nonlife Insurance Company A and Nonlife Insurance Agency B in order to check its effectiveness

Faunal make-up, host range and infestation rate of weevils and tephritid flies associated with flower heads of the thistle Cirsium (Cardueae : Astaraceae) in Japan

金沢大学環日本海域環境研究センター生物多様性研究部門From 1988 to 1998, we collected flower heads of 39 thistle taxa (35 taxa of Cirsium, one species each of Breea, Synurus, Saussurea and Arctium; Cardueae; Astaraceae) in Japan, mainly from Hokuriku and other parts of central Honshu, and kept them in the laboratory to breed weevils and tephritid flies, the core fauna. We report the faunal make-up, host plants, geographic distribution and the attack levels of the insects. Results indicated that (i) three Larinus species (Curculionidae) and three species of tephritid flies (Tephritis, Urophora and Xyphosia) comprised the core fauna; (ii) two insect species belonging to the same taxonomic group (either Curculionidae (Larinus) or Tephritidae) tended to use different host plant species; (iii) two sympatric Larinus species (L. latissimus and L. meleagris) segregated the host plants seasonally in central Honshu (Cirsium blooming in spring and autumn, respectively); and (iv) two tephritid fly species, Xyphosia punctigera and Urophora sachalinensis, segregated geographically (the former on the Japan Sea side and the latter on the Pacific Ocean side). In comparison with their European counterparts, the weevils and tephritids of the Japanese Cirsium are characterized by a lower species richness and a lower degree of specialization in usage of the thistle flower heads, with gall-formers being distinctly under-represented, and callus tissue-feeders being absent. This reflects the fact that Japanese thistles are so closely related that hybridization frequently occurs, and also that the thistles have had a short history of interaction with the insects since the thistles’ arrival in Japan.Embargo Period 24 month

Demography of the Heike firefly Luciola lateralis (Coleoptera: Lampyridae), a representative species of Japan\u27s traditional agricultural landscape

Populations of the Heike firefly, Luciola lateralis, a representative species of Japan\u27s traditional agricultural landscape (known as satoyama), have recently experienced rapid declines in many areas of Japan. Owing to the popularity of this firefly, many local communities have increased conservation efforts through the restoration of aquatic habitat complexes in satoyama. To provide fundamental parameters to predict population dynamics of the firefly, we conducted a mark-recapture study in restored paddy fields, and we estimated adult population parameters such as population size, survival, recruitment, sex ratio, and body size. We found that capture probability generally decreased as the season advanced, probably because of seasonal changes in detectability and/or firefly behavior. The daily survival rate of adults decreased over the season and may be related to a seasonal decline in adult body size. Adult population exhibited a highly male-biased sex ratio. Firefly abundance in the restored paddy fields doubled during the 4-year study period. Our analysis showed that adult detectability, recruitment, and survival rate are seasonally variable and could affect population size estimates obtained by a simple flash census. The mark-recapture technique can provide precise estimates of adult L. lateralis population characteristics and, thus, is a valuable method for predicting firefly populations and assessing the success of the restoration program. © 2012 Springer Science+Business Media B.V

Increasing Incidence and Age at Onset of Amyotrophic Lateral Sclerosis in Nagano Prefecture, Japan

Article信州医学雑誌 64(5): 239-246(2016)journal articl

Examining the Validity of Efforts of the Muscular Dystrophy Medical Network in Nagano : A Questionnaire Study

Article信州医学雑誌 66(3): 205-212(2018)journal articl

Matrix metalloproteinase-2 ablation in dystrophin-deficient mdx muscles reduces angiogenesis resulting in impaired growth of regenerated muscle fibers

Matrix metalloproteases (MMPs) are a family of endopeptidases classified into subgroups based on substrate preference in normal physiological processes such as embryonic development and tissue remodeling, as well as in various disease processes via degradation of extracellular matrix components. Among the MMPs, MMP-9 and MMP-2 have been reported to be up-regulated in skeletal muscles in the lethal X-linked muscle disorder Duchenne muscular dystrophy (DMD), which is caused by loss of dystrophin. A recent study showed that deletion of the MMP9 gene in mdx, a mouse model for DMD, improved skeletal muscle pathology and function; however, the role of MMP-2 in the dystrophin-deficient muscle is not well known. In this study, we aimed at verifying the role of MMP-2 in the dystrophin-deficient muscle by using mdx mice with genetic ablation of MMP-2 (mdx/MMP-2(-/-)). We found impairment of regenerated muscle fiber growth with reduction of angiogenesis in mdx/MMP-2(-/-) mice at 3 months of age. Expression of vascular endothelial growth factor-A (VEGF-A), an important angiogenesis-related factor, decreased in mdx/MMP-2(-/-) mice at 3 months of age. MMP-2 had not a critical role in the degradation of dystrophin-glycoprotein complex (DGC) components such as beta-dystroglycan and beta-sarcoglycan in the regeneration process of the dystrophic muscle. Accordingly, MMP-2 may be essential for growth of regenerated muscle fibers through VEGF-associated angiogenesis in the dystrophin-deficient skeletal muscle.ArticleHUMAN MOLECULAR GENETICS. 20(9):1787-1799 (2011)journal articl