Toya Ohashi

Summary

Affiliation: Jikei University School of Medicine
Country: Japan

Publications

  1. doi request reprint Immunogene therapy against colon cancer metastasis using an adenovirus vector expressing CD40 ligand
    Tomonori Iida
    Department of Surgery, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Surgery 148:925-35. 2010
  2. ncbi request reprint Enzyme replacement therapy for lysosomal storage diseases
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Pediatr Endocrinol Rev 10:26-34. 2012
  3. pmc Administration of anti-CD3 antibodies modulates the immune response to an infusion of α-glucosidase in mice
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, Research Center for Medical Sciences, The Jikei University School of Medicine, Tokyo, Japan
    Mol Ther 20:1924-31. 2012
  4. ncbi request reprint Reduced alpha-Gal A enzyme activity in Fabry fibroblast cells and Fabry mice tissues induced by serum from antibody positive patients with Fabry disease
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minatoku, Tokyo 105 8461, Japan
    Mol Genet Metab 94:313-8. 2008
  5. ncbi request reprint Influence of antibody formation on reduction of globotriaosylceramide (GL-3) in urine from Fabry patients during agalsidase beta therapy
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minatoku, Tokyo 105 8461, Japan
    Mol Genet Metab 92:271-3. 2007
  6. doi request reprint Inhibition of nuclear factor kappa-B enhances the antitumor effect of combination treatment with tumor necrosis factor-alpha gene therapy and gemcitabine for pancreatic cancer in mice
    Yuki Fujiwara
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    J Am Coll Surg 216:320-32.e3. 2013
  7. ncbi request reprint Widespread and highly persistent gene transfer to the CNS by retrovirus vector in utero: implication for gene therapy to Krabbe disease
    Jin Song Shen
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    J Gene Med 7:540-51. 2005
  8. doi request reprint Adenovirus-mediated CD40L gene therapy induced both humoral and cellular immunity against rat model of hepatocellular carcinoma
    Tomonori Iida
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    Cancer Sci 99:2097-103. 2008
  9. doi request reprint Inhibition of nuclear factor-κB enhances the antitumor effect of paclitaxel against gastric cancer with peritoneal dissemination in mice
    Koichiro Haruki
    Department of Surgery, The Jikei University School of Medicine, 3 25 8, Nishi Shinbashi, Minato ku, Tokyo, 105 8461, Japan
    Dig Dis Sci 58:123-31. 2013
  10. doi request reprint Combination treatment using adenovirus vector-mediated tumor necrosis factor-alpha gene transfer and a NF-κB inhibitor for pancreatic cancer in mice
    Kenei Furukawa
    Department of Surgery, Jikei University School of Medicine, Tokyo, Japan
    Cancer Lett 306:92-8. 2011

Detail Information

Publications66

  1. doi request reprint Immunogene therapy against colon cancer metastasis using an adenovirus vector expressing CD40 ligand
    Tomonori Iida
    Department of Surgery, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Surgery 148:925-35. 2010
    ..We studied CD40 ligand (CD40L)-mediated immunogene therapy for metastatic liver cancer in rats...
  2. ncbi request reprint Enzyme replacement therapy for lysosomal storage diseases
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Pediatr Endocrinol Rev 10:26-34. 2012
    ..Finally, the development of a new enzyme that effectively targets the brain and bone is very important to improve the quality of life of patients with LSDs...
  3. pmc Administration of anti-CD3 antibodies modulates the immune response to an infusion of α-glucosidase in mice
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, Research Center for Medical Sciences, The Jikei University School of Medicine, Tokyo, Japan
    Mol Ther 20:1924-31. 2012
    ..This suggests that CD4(+)CD25(+) cells are important for the immune suppressive activity of anti-CD3 Abs. In summary, anti- CD3 Abs are useful for inducing immune tolerance to ERT for PD...
  4. ncbi request reprint Reduced alpha-Gal A enzyme activity in Fabry fibroblast cells and Fabry mice tissues induced by serum from antibody positive patients with Fabry disease
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minatoku, Tokyo 105 8461, Japan
    Mol Genet Metab 94:313-8. 2008
    ....
  5. ncbi request reprint Influence of antibody formation on reduction of globotriaosylceramide (GL-3) in urine from Fabry patients during agalsidase beta therapy
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minatoku, Tokyo 105 8461, Japan
    Mol Genet Metab 92:271-3. 2007
    ..Normalization of uGL-3 was achieved more efficiently in seronegatives. But different from previous reports, reduction of uGL-3 level was observed in some seropositive patients...
  6. doi request reprint Inhibition of nuclear factor kappa-B enhances the antitumor effect of combination treatment with tumor necrosis factor-alpha gene therapy and gemcitabine for pancreatic cancer in mice
    Yuki Fujiwara
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    J Am Coll Surg 216:320-32.e3. 2013
    ..We hypothesized that nafamostat mesilate (FUT175), an NF-κB inhibitor, enhances the antitumor effect of combination treatment with an adenoviral vector-expressing TNF-α (AxCAhTNF-α) and gemcitabine for pancreatic cancer in mice...
  7. ncbi request reprint Widespread and highly persistent gene transfer to the CNS by retrovirus vector in utero: implication for gene therapy to Krabbe disease
    Jin Song Shen
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    J Gene Med 7:540-51. 2005
    ..In this study, in order to obtain permanent gene transduction, we investigated the feasibility of retrovirus-mediated in utero gene transduction...
  8. doi request reprint Adenovirus-mediated CD40L gene therapy induced both humoral and cellular immunity against rat model of hepatocellular carcinoma
    Tomonori Iida
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    Cancer Sci 99:2097-103. 2008
    ..These observations suggested that CD40L-mediated immune gene therapy for HCC was very effective treatment by activation of both cellular and humoral immune system...
  9. doi request reprint Inhibition of nuclear factor-κB enhances the antitumor effect of paclitaxel against gastric cancer with peritoneal dissemination in mice
    Koichiro Haruki
    Department of Surgery, The Jikei University School of Medicine, 3 25 8, Nishi Shinbashi, Minato ku, Tokyo, 105 8461, Japan
    Dig Dis Sci 58:123-31. 2013
    ..Chemoresistance due to paclitaxel-induced nuclear factor-kappa B (NF-κB) activation is an important cause of suboptimal therapeutic efficacy...
  10. doi request reprint Combination treatment using adenovirus vector-mediated tumor necrosis factor-alpha gene transfer and a NF-κB inhibitor for pancreatic cancer in mice
    Kenei Furukawa
    Department of Surgery, Jikei University School of Medicine, Tokyo, Japan
    Cancer Lett 306:92-8. 2011
    ..05). In conclusion, adenovirus vector-mediated TNF-α gene therapy combined with nafamostat mesilate was effective for pancreatic cancer in mice...
  11. doi request reprint Combination chemotherapy of nafamostat mesylate with gemcitabine for gallbladder cancer targeting nuclear factor-κB activation
    Ryota Iwase
    Department of Surgery, Jikei University School of Medicine, Tokyo, Japan
    J Surg Res 184:605-12. 2013
    ..Therefore, we hypothesized that nafamostat mesylate inhibits gemcitabine-induced NF-κB activation and enhances apoptosis induced by gemcitabine in gallbladder cancer...
  12. doi request reprint Combination paclitaxel and inhibitor of nuclear factor κB activation improves therapeutic outcome for model mice with peritoneal dissemination of pancreatic cancer
    Yuki Fujiwara
    Department of Surgery, Institute of DNA Medicine, Jikei University School of Medicine, Minato ku, Tokyo, Japan
    Pancreas 40:600-7. 2011
    ..We hypothesized that addition of FUT175 to PTX may enhance the antitumor effect in peritoneal dissemination of pancreatic cancer...
  13. doi request reprint Combination chemotherapy of serine protease inhibitor nafamostat mesilate with oxaliplatin targeting NF-κB activation for pancreatic cancer
    Takeshi Gocho
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    Cancer Lett 333:89-95. 2013
    ..Combination chemotherapy using nafamostat mesilate and oxaliplatin induces synergistic cytotoxicity in pancreatic cancer and could be a novel strategy for treatment...
  14. doi request reprint Akt inactivation induces endoplasmic reticulum stress-independent autophagy in fibroblasts from patients with Pompe disease
    Yurika Nishiyama
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo 105 8461, Japan
    Mol Genet Metab 107:490-5. 2012
    ....
  15. doi request reprint Endoplasmic reticulum stress induces autophagy through activation of p38 MAPK in fibroblasts from Pompe disease patients carrying c.546G>T mutation
    Yohta Shimada
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 104:566-73. 2011
    ....
  16. ncbi request reprint Adenovirus vector-mediated gene therapy using iodized oil esters for hepatocellular carcinoma in rats
    Hiroaki Shiba
    Department of Surgery, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Anticancer Res 28:51-3. 2008
    ..The usefulness of gene therapy by intraarterial injection of an adenovirus vector with iodized oil esters (IOEs) for hepatocellular carcinoma (HCC) was studied...
  17. doi request reprint Morphological features of iPS cells generated from Fabry disease skin fibroblasts using Sendai virus vector (SeVdp)
    Shiho Kawagoe
    Department of Genetic Diseases and Genomic Science, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 109:386-9. 2013
    ..because of a massive accumulation of membranous cytoplasmic bodies in lysosomes, possibly the persistent damages of intracellular architecture. ..
  18. doi request reprint Nuclear factor κB activity correlates with the progression and prognosis of pancreatic cancer in a mouse model
    Kenei Furukawa
    Department of Surgery, Jikei University School of Medicine, 3 25 8 Nishi shinbashi, Minato ku, Tokyo 105 8461, Japan
    Surg Today 43:171-7. 2013
    ..In this study, we quantitated NF-κB activity of various human pancreatic cancer cell lines and evaluated whether NF-κB activity was related to tumor progression and prognosis for pancreatic cancer in mice...
  19. ncbi request reprint A novel approach for gene transduction with adenovirus vector and the fibrin glue system
    Minori Sakurai
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    Anticancer Res 28:3809-13. 2008
    ..In this study, as a novel and unique approach, the possibility and efficacy of locoregional gene transfer using the FGS containing an adenoviral vector was determined...
  20. doi request reprint A thermoreversible polymer mediates controlled release of glial cell line-derived neurotrophic factor to enhance kidney regeneration
    Yousof Gheisari
    Project Laboratory for Kidney Regeneration, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Artif Organs 34:642-7. 2010
    ..These findings suggest that the GDNF-polymer can be used as a diffuser of GDNF for kidney organogenesis...
  21. ncbi request reprint GALC transduction leads to morphological improvement of the twitcher oligodendrocytes in vivo
    Xing Li Meng
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 84:332-43. 2005
    ....
  22. doi request reprint Non-invasive high-risk screening for Fabry disease hemizygotes and heterozygotes
    Teruo Kitagawa
    Tokyo Health Service Association, 1 2, Sadohara cho, Ichigaya, Sinjuku ku, Tokyo, 162 8402, Japan
    Pediatr Nephrol 23:1461-71. 2008
    ..However, we recommend that if the results of the first measurements exceed the cut-off values for only one of these biomarkers, another urine sample should be requested for re-assay to confirm the result...
  23. ncbi request reprint In vitro and pathological investigations of MODY5 with the R276X-HNF1beta (TCF2) mutation
    Kei Fujimoto
    Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan
    Endocr J 54:757-64. 2007
    ..Analysis with light and electron microscopy on biopsied kidney specimens suggested that the origin of the cysts might be glomeruli but the primary lesion could be tubules...
  24. doi request reprint Perioperative change in white blood cell count predicts outcome of hepatic resection for hepatocellular carcinoma
    Yuki Fujiwara
    Department of Surgery, Jikei University School of Medicine, 3 25 8 Nishi shinbashi, Minato ku, Tokyo, 105 8461, Japan
    J Hepatobiliary Pancreat Sci 17:892-7. 2010
    ..Therefore, we investigated the relation between perioperative change in white blood cell count (WBC) and tumor recurrence as well as survival in patients with HCC after hepatic resection...
  25. doi request reprint Nafamostat mesilate can prevent adhesion, invasion and peritoneal dissemination of pancreatic cancer thorough nuclear factor kappa-B inhibition
    Yuki Fujiwara
    Department of Surgery, The Jikei University School of Medicine, 3 25 8, Nishi Shinbashi, Minato ku, Tokyo 105 8461, Japan
    J Hepatobiliary Pancreat Sci 18:731-9. 2011
    ..We hypothesized that nafamostat mesilate may inhibit cell adhesion, angiogenesis, invasion and metastases in peritoneal dissemination of pancreatic cancer...
  26. doi request reprint Inhibition of nuclear factor-κB enhances the antitumor effect of tumor necrosis factor-α gene therapy for hepatocellular carcinoma in mice
    Koichiro Haruki
    Department of Surgery, The Jikei University School of Medicine, Tokyo, Japan
    Surgery 154:468-78. 2013
    ..We hypothesized that the NF-κB inhibitor nafamostat mesilate would enhance the antitumor effect of adenovirus vector-mediated TNF-α gene therapy for HCC...
  27. doi request reprint No accumulation of globotriaosylceramide in the heart of a patient with the E66Q mutation in the α-galactosidase A gene
    Masahisa Kobayashi
    Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 107:711-5. 2012
    ..However, no pathological study was reported. We carried out pathological studies using a cardiac biopsy specimen from a patient with the E66Q mutation...
  28. doi request reprint Minimum requirement of donor cells to reduce the glycolipid storage following bone marrow transplantation in a murine model of Fabry disease
    Takayuki Yokoi
    Department of Gene Therapy, Institute of DNA Medicine Higuchi, The Jikei University School of Medicine, Tokyo, Japan
    J Gene Med 13:262-8. 2011
    ....
  29. ncbi request reprint Immunogene therapy by adenovirus vector expressing CD40 ligand for metastatic liver cancer in rats
    Ken Hanyu
    Department of Surgery, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Anticancer Res 28:2785-9. 2008
    ..We have explored a gene-therapeutic approach to stimulate antitumor immunity by adenoviral-mediated transfer of CD40 ligand (CD40L) to treat metastatic liver cancer in a rat model...
  30. doi request reprint Enzyme replacement therapy (ERT) procedure for mucopolysaccharidosis type II (MPS II) by intraventricular administration (IVA) in murine MPS II
    Takashi Higuchi
    Department of Genetic Diseases and Genomic Science, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 107:122-8. 2012
    ..Loss of vacuoles was also observed in other tissues (liver, kidney and testis). These results demonstrate the possible efficacy of an ERT procedure with intraventricular administration of IDS for the treatment of MPS II...
  31. ncbi request reprint Anti-tumor effect by inhibition of NF-kappaB activation using nafamostat mesilate for pancreatic cancer in a mouse model
    Kenei Furukawa
    Jikei University School of Medicine, Minato ku, Tokyo 105 8461, Japan
    Oncol Rep 24:843-50. 2010
    ..These results suggested that nafamostat mesilate has anti-neoplastic property against experimental pancreatic cancer...
  32. ncbi request reprint The generation of induced pluripotent stem cells (iPSCs) from patients with infantile and late-onset types of Pompe disease and the effects of treatment with acid-α-glucosidase in Pompe's iPSCs
    Takashi Higuchi
    Department of Genetic Diseases and Genomic Science, The Jikei University School of Medicine, Tokyo, Japan Department of Gene Therapy, Research Center for Medical Sciences, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 112:44-8. 2014
    ..It represents a promising resource to study disease mechanisms, screen new drug compounds and develop new therapies for Pompe disease. ..
  33. pmc Induced pluripotent stem cells derived from mouse models of lysosomal storage disorders
    Xing Li Meng
    Department of Gene Therapy, Jikei University School of Medicine, Tokyo 105 8461, Japan
    Proc Natl Acad Sci U S A 107:7886-91. 2010
    ..Thus, our study demonstrates the unique promise of iPS cells for studying the pathogenesis and treatment of LSDs...
  34. ncbi request reprint Generation of a transplantable erythropoietin-producer derived from human mesenchymal stem cells
    Takashi Yokoo
    Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Transplantation 85:1654-8. 2008
    ..Together these results confirmed the generation of a stem cell-derived organoid that is capable of producing EPO and sensitive to physiological regulation...
  35. ncbi request reprint Brain transplantation of genetically engineered human neural stem cells globally corrects brain lesions in the mucopolysaccharidosis type VII mouse
    Xing Li Meng
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    J Neurosci Res 74:266-77. 2003
    ....
  36. pmc Human mesenchymal stem cells in rodent whole-embryo culture are reprogrammed to contribute to kidney tissues
    Takashi Yokoo
    Department of Internal Medicine and Gene Therapy and Pediatrics, Institute of DNA Medicine, Jikei University School of Medicine, 3 25 8, Nishi Shimbashi, Minato ku, Tokyo 105 8461, Japan
    Proc Natl Acad Sci U S A 102:3296-300. 2005
    ..We show that a combination of whole-embryo culture, followed by organ culture, encourages exogenous human mesenchymal stem cells to differentiate and contribute to functional complex structures of the new kidney...
  37. ncbi request reprint Xenobiotic kidney organogenesis from human mesenchymal stem cells using a growing rodent embryo
    Takashi Yokoo
    Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, 3 25 8 Nishi shimbashi, Minato ku, Tokyo, Japan 105 8461
    J Am Soc Nephrol 17:1026-34. 2006
    ..1 versus 0.3 +/- 0.2 mg, respectively), suggesting that the neo-kidney may produce urine. Taken together, these findings suggest that hMSC can differentiate into a mature renal structure with the potential to replace lost kidney function...
  38. doi request reprint Generation of induced pluripotent stem (iPS) cells derived from a murine model of Pompe disease and differentiation of Pompe-iPS cells into skeletal muscle cells
    Shiho Kawagoe
    Department of Genetic Diseases and Genomic Science, The Jikei University School of Medicine, Tokyo, Japan
    Mol Genet Metab 104:123-8. 2011
    ..This study indicates that the iPS and skeletal muscle cells generated in this study could also be a useful disease model for studies investigating the pathogenesis and treatment of skeletal muscle in Pompe disease...
  39. ncbi request reprint Widespread gene transduction to the central nervous system by adenovirus in utero: implication for prenatal gene therapy to brain involvement of lysosomal storage disease
    Jin Song Shen
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Minato ku, Tokyo 105 8461, Japan
    J Gene Med 6:1206-15. 2004
    ..The aim of this study is to investigate the feasibility and effectiveness of brain-directed prenatal gene therapy for lysosomal storage diseases...
  40. doi request reprint Pathology of the first autopsy case diagnosed as mucolipidosis type III α/β suggesting autophagic dysfunction
    Hiroshi Kobayashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Japan
    Mol Genet Metab 102:170-5. 2011
    ..This pathological study of MLIII supports the theory that MLIII is a mild type of MLII because of the close similarity of their pathological findings...
  41. doi request reprint Proteasome inhibitors improve the function of mutant lysosomal α-glucosidase in fibroblasts from Pompe disease patient carrying c.546G>T mutation
    Yohta Shimada
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo 105 8461, Japan
    Biochem Biophys Res Commun 415:274-8. 2011
    ..These findings indicate that proteasome inhibitor may be a novel drug as potential pharmacological chaperone therapy for Pompe disease patient carrying chaperon-responsive mutation...
  42. doi request reprint Inner ear pathology of alpha-galactosidase A deficient mice, a model of Fabry disease
    Yuika Sakurai
    Department of Otorhinolarygology, The Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minato ku, Tokyo, Japan
    Auris Nasus Larynx 37:274-80. 2010
    ..Accordingly, histopathological studies of the cochlea were performed on an alpha-galactosidase A deficient murine model of Fabry disease, using C57BL6/J mice as the controls...
  43. doi request reprint Enzyme augmentation therapy enhances the therapeutic efficacy of bone marrow transplantation in mucopolysaccharidosis type II mice
    Kazumasa Akiyama
    Department of Gene Therapy, Institute of DNA Medicine, Research Center for Medical Sciences, The Jikei University School of Medicine, Tokyo, Japan Department of Pediatrics, Kitasato University Graduate School of Medicine, Kanagawa, Japan
    Mol Genet Metab 111:139-46. 2014
    ..Finally, we showed that pathologic GAGs might potentially represent a sensitive biomarker to monitor the therapeutic efficacy of therapies for MPS II...
  44. ncbi request reprint [Gene therapy for lysosomal disorders]
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine
    Nihon Rinsho 63:671-8. 2005
  45. ncbi request reprint Antitumor immunity against bladder cancer induced by ex vivo expression of CD40 ligand gene using retrovirus vector
    Takahiro Kimura
    Department of Urology, The Jikei University School of Medicine, 3 25 8 Nishi shinbashi, Minato ku, Tokyo, Japan
    Cancer Gene Ther 10:833-9. 2003
    ..These data demonstrated that the antitumor immunity induced by CD40L was effective in the vaccination model and suggested that immunogene therapy using CD40L may be a new strategy of systemic therapy against bladder cancer...
  46. ncbi request reprint Non-invasive screening method for Fabry disease by measuring globotriaosylceramide in whole urine samples using tandem mass spectrometry
    Teruo Kitagawa
    Tokyo Health Service Association, Tokyo, Japan
    Mol Genet Metab 85:196-202. 2005
    ..We propose that this procedure can be used as a reliable, non-invasive, simple method for general and high-risk population screening for hemizygotic patients with the classic type and probably renal variant of Fabry disease...
  47. doi request reprint Functional development of a transplanted embryonic kidney: effect of transplantation site
    Kei Matsumoto
    Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Minato ku, Tokyo, Japan
    J Nephrol 25:50-5. 2012
    ..Thus, we determined the optimal transplantation site for the metanephros in terms of retaining its ability to produce renin and erythropoietin (EPO)...
  48. ncbi request reprint Systemic accumulation of undigested lysosomal metabolites in an autopsy case of mucolipidosis type II; autophagic dysfunction in cardiomyocyte
    Yohei Sato
    Department of Pediatrics, The Jikei University School of Medicine, Japan Department of Gene Therapy, Institute of DNA Medicine, Research Center for Medical Sciences, The Jikei University School of Medicine, Japan Electronic address
    Mol Genet Metab 112:224-8. 2014
    ..Ubiquitin, p62, K48 polyubiquitin, and K63 polyubiquitin were also positive in the cytoplasm. Our findings suggest that autophagic dysfunction might be associated with the cardiomyopahty of mucolipidosis type II. ..
  49. ncbi request reprint Significance of screening for Fabry disease among male dialysis patients
    Mayuri Ichinose
    Division of Kidney and Hypertension, Department of Internal Medicine, Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minato ku, Tokyo, 105 8461, Japan
    Clin Exp Nephrol 9:228-32. 2005
    ..In this study, we clarified the incidence of this atypical variant of Fabry disease in hemodialysis patients...
  50. pmc Genetic association of glutathione peroxidase-1 with coronary artery calcification in type 2 diabetes: a case control study with multi-slice computed tomography
    Masami Nemoto
    Division of Diabetes, Metabolism and Endocrinology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan
    Cardiovasc Diabetol 6:23. 2007
    ..This study evaluated the effect of genetic polymorphism of enzymes producing or responsible for reducing ROS on coronary artery calcification in type 2 diabetes (T2D)...
  51. ncbi request reprint Adenovirus vector-mediated gene transfer using degradable starch microspheres for hepatocellular carcinoma in rats
    Hiroaki Shiba
    Department of Surgery, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    J Surg Res 133:193-6. 2006
    ..Therefore, we studied the use of DSM for adenovirus vector-mediated gene transfer to HCC in vivo...
  52. ncbi request reprint OAT mutations and clinical features in two Japanese brothers with gyrate atrophy of the choroid and retina
    Satoshi Katagiri
    Departments of Ophthalmology, The Jikei University School of Medicine, 3 25 8 Nishi shimbashi, Minato ku, Tokyo, 105 8461, Japan
    Doc Ophthalmol 128:137-48. 2014
    ..The purpose of this study was to report a novel deletion mutation of the OAT gene and describe clinical features of two brothers with GA in a Japanese family...
  53. doi request reprint Novel interferon-based pre-transplantation conditioning in the treatment of a congenital metabolic disorder
    Taku Sato
    Department of Biodefense Research, Medical Research Institute, Tokyo Medical and Dental University, Tokyo 113 5810, Japan
    Blood 121:3267-73. 2013
    ..Our findings suggest type I IFN-based preconditioning, combined with HSC transplantation, as a novel nongenotoxic treatment of some congenital diseases...
  54. doi request reprint Prognostic factors for the late onset Pompe disease with enzyme replacement therapy: from our experience of 4 cases including an autopsy case
    Hiroshi Kobayashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, 3 5 28 Nishi Shinbashi, Minato ku, Tokyo, Japan
    Mol Genet Metab 100:14-9. 2010
    ....
  55. doi request reprint Comparison of multipotency and molecular profile of MSCs between CKD and healthy rats
    Akifumi Yamada
    Project Laboratory for Kidney Regeneration, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Hum Cell 27:59-67. 2014
    ..However, long-term exposure to uremic toxin and the differences in gene expression of MSCs derived from bone marrow or adipose tissue may affect renal regeneration. ..
  56. ncbi request reprint Gene delivery using human cord blood-derived CD34+cells into inflamed glomeruli in NOD/SCID mice
    Takashi Yokoo
    Division of Nephrology and Hypertension, Department of Internal Medicine, Institute of DNA Medicine, Jikei University School of Medicine, Tokyo, Japan
    Kidney Int 64:102-9. 2003
    ..In this regard, umbilical cord blood may be a useful alternative and, therefore, we focused on their suitability as a source of hematopoietic stem cells for transplantation-based therapy for glomerulonephritis...
  57. doi request reprint Gaucher disease patient with myoclonus epilepsy and a novel mutation
    Asako Tajima
    Department of Pediatrics, Jikei University School of Medicine, Tokyo 105 8461, Japan
    Pediatr Neurol 42:65-8. 2010
    ..4%. Neither mutation influenced the stability of the enzyme protein. These data suggested a diagnosis of Gaucher disease for this patient, and indicated that G199D is a novel mutation...
  58. ncbi request reprint Enhanced insulin secretion from engineered 3T3-L1 preadipocytes by induction of cellular differentiation
    Kei Fujimoto
    Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan
    Mol Cell Biochem 268:1-8. 2005
    ..Results of our present study will provide important information on cell-based therapy using undifferentiated progenitors and tissue stem cells...
  59. ncbi request reprint Glasgow prognostic score is related to blood transfusion requirements and post-operative complications in hepatic resection for hepatocellular carcinoma
    Yuki Fujiwara
    Department of Surgery, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    Anticancer Res 30:5129-36. 2010
    ..The aim of this study was to evaluate the significance GPS in hepatic resection for hepatocellular carcinoma (HCC)...
  60. doi request reprint The hearing status in 12 female and 15 male Japanese Fabry patients
    Yuika Sakurai
    Department of Otorhinolaryngology, The Jikei University School of Medicine, 3 25 8 Nishishinbashi, Minato ku, Tokyo 105 8461, Japan
    Auris Nasus Larynx 36:627-32. 2009
    ..Accordingly, we examined the hearing status of both male and female FD patients and elucidated the similarities and differences. We also analyzed for correlations between the hearing status and renal and heart disorders...
  61. ncbi request reprint Adenoviral gene transfer of GDNF, BDNF and TGF beta 2, but not CNTF, cardiotrophin-1 or IGF1, protects injured adult motoneurons after facial nerve avulsion
    Tsuyoshi Sakamoto
    Department of Molecular Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Tokyo, Japan
    J Neurosci Res 72:54-64. 2003
    ..These results indicate that the gene transfer of GDNF and BDNF and TGFbeta2 but not CNTF, CT1 or IGF1 may prevent the degeneration of motoneurons in adult humans with motoneuron injury and motor neuron diseases...
  62. ncbi request reprint Human Shugoshin mediates kinetochore-driven formation of kinetochore microtubules
    Hideaki Suzuki
    Department of Gene Therapy, Institute of DNA Medicine, Department of Pediatrics, Institute of DNA Medicine, The Jikei University School of Medicine, Nishi Shinbashi, Minato ku, Tokyo, Japan
    Cell Cycle 5:1094-101. 2006
    ..Interestingly, we also found that hSgo is rapidly exchanged at kinetochores at early mitosis. Based on these results, we conclude that hSgo may be diffusible and have a role in proper kinetochores MTs attachment...
  63. ncbi request reprint Allogenic bone marrow transplantation for late-infantile neuronal ceroid lipofuscinosis
    Yuki Yuza
    Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan
    Pediatr Int 47:681-3. 2005
  64. ncbi request reprint Establishment and characterization of spontaneously immortalized Schwann cells from murine model of globoid cell leukodystrophy (twitcher)
    Jin Song Shen
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine, Tokyo, Japan
    J Neurosci Res 68:588-94. 2002
    ..These immortalized Schwann cells can be useful in studies on the nervous system lesions in GLD...
  65. ncbi request reprint [Gene analysis of Gaucher disease]
    Toya Ohashi
    Department of Gene Therapy, Institute of DNA Medicine, The Jikei University School of Medicine
    Nihon Rinsho 63:498-500. 2005
    ..Depends of presence of neurological symptoms, onset of disease and severity of disease, the disease is divided into three phenotypes. More than 180 mutations were reported and phenotype/genotype relations was somehow elucidated...
  66. ncbi request reprint Novel treatment for neuronopathic lysosomal storage diseases--cell therapy/gene therapy
    Yoshikatsu Eto
    Department of Pediatrics, Institute for DNA Medicine, Tokyo Jikei University School of Medicine, Japan
    Curr Mol Med 2:83-9. 2002
    ..Additional approaches have included substrate deprivation/chaperone therapy for the treatment of LSD. This article reviews these new technologies, discusses recent progress, and suggests their possible application...