Junko Matsuda

Summary

Affiliation: The University of Tokushima
Country: Japan

Publications

  1. ncbi request reprint Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse
    Junko Matsuda
    Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, 3 18 15, Kuramoto Cho, Tokushima 770 8503, Japan
    Hum Mol Genet 13:2709-23. 2004
  2. ncbi request reprint Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the "twitcher" mouse model of globoid cell leukodystrophy
    Kumiko Tominaga
    Department of Pediatrics, University of Tokushima School of Medicine, Tokushima, Japan
    J Neurosci Res 77:507-16. 2004
  3. ncbi request reprint Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region
    Etsuo Naito
    Department of Pediatrics, School of Medicine, University of Tokushima, Kuramoto Cho 3, Tokushima, Japan
    Biochim Biophys Acta 1588:79-84. 2002
  4. ncbi request reprint Plasma adiponectin levels in newborns are higher than those in adults and positively correlated with birth weight
    Yumiko Kotani
    Department of Pediatrics, School of Medicine, University of Tokushima, 3 Kuramoto cho, Japan
    Clin Endocrinol (Oxf) 61:418-23. 2004
  5. doi request reprint Successful allogeneic unrelated bone marrow transplantation using reduced-intensity conditioning for the treatment of X-linked adrenoleukodystrophy in a one-yr-old boy
    Kazumi Okamura
    Department of Pediatrics, University of Tokushima Graduate School of Medical Science, Tokushima, Japan
    Pediatr Transplant 13:130-3. 2009
  6. ncbi request reprint Comparative clinico-pathological study of saposin-A-deficient (SAP-A-/-) and Twitcher mice
    Takashi Yagi
    Department of Pathology and Laboratory Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599 7525, USA
    J Neuropathol Exp Neurol 63:721-34. 2004
  7. ncbi request reprint Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice
    Keiko Tadano-Aritomi
    Department of Biochemistry, Teikyo University School of Medicine, Kaga 2 11 1, Itabashi ku, Tokyo 173 8605, Japan
    J Lipid Res 44:1737-43. 2003
  8. ncbi request reprint Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking
    Ying Sun
    Division of Human Genetics, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Hum Mol Genet 16:957-71. 2007
  9. ncbi request reprint Hematopoietic cell transplantation ameliorates clinical phenotype and progression of the CNS pathology in the mouse model of late onset Krabbe disease
    Takashi Yagi
    Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA
    J Neuropathol Exp Neurol 64:565-75. 2005
  10. ncbi request reprint The function of sphingolipids in the nervous system: lessons learnt from mouse models of specific sphingolipid activator protein deficiencies
    Junko Matsuda
    Institute of Glycotechnology, Future Science and Technology Joint Research Center, Tokai University, Hiratsuka, Japan
    J Neurochem 103:32-8. 2007

Collaborators

  • Ichiro Yokota
  • Kunihiko Suzuki
  • Eiji Takeda
  • G A Grabowski
  • Ying Sun
  • Takashi Yagi
  • Etsuo Naito
  • Kumiko Tominaga
  • Yasuhiro Kuroda
  • Kazumi Okamura
  • Yumiko Kotani
  • Seiko Kitamura
  • Keiko Tadano-Aritomi
  • Toshihiro Onishi
  • Kenji Mori
  • Emiko Fujii
  • Tsutomu Watanabe
  • Hiroyoshi Watanabe
  • Kazunori Toida
  • Shoichi Takikita
  • Makiko Kido
  • Ikuko Mohri
  • Kazunori Ishimura
  • Hirokazu Fujimoto
  • Ineo Ishizuka
  • Michinori Ito
  • Yukiko Ogawa
  • Yoshihiro Horii
  • Takahiko Saijo
  • Eiko Takada

Detail Information

Publications10

  1. ncbi request reprint Mutation in saposin D domain of sphingolipid activator protein gene causes urinary system defects and cerebellar Purkinje cell degeneration with accumulation of hydroxy fatty acid-containing ceramide in mouse
    Junko Matsuda
    Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, 3 18 15, Kuramoto Cho, Tokushima 770 8503, Japan
    Hum Mol Genet 13:2709-23. 2004
    ..These results not only indicate the role of saposin D in in vivo ceramide metabolism, but also suggest possible cytotoxicity of ceramide underlying the cerebellar Purkinje cell and renal tubular cell degeneration...
  2. ncbi request reprint Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the "twitcher" mouse model of globoid cell leukodystrophy
    Kumiko Tominaga
    Department of Pediatrics, University of Tokushima School of Medicine, Tokushima, Japan
    J Neurosci Res 77:507-16. 2004
    ..These findings also suggest an intriguing possibility of important function of some sphingolipids in the hippocampal neuronal organization and maintenance...
  3. ncbi request reprint Thiamine-responsive pyruvate dehydrogenase deficiency in two patients caused by a point mutation (F205L and L216F) within the thiamine pyrophosphate binding region
    Etsuo Naito
    Department of Pediatrics, School of Medicine, University of Tokushima, Kuramoto Cho 3, Tokushima, Japan
    Biochim Biophys Acta 1588:79-84. 2002
    ..The DNA sequence of these two male patients' X-linked E1alpha subunit revealed a point mutation (F205L and L216F) within the TPP-binding region in exon 7...
  4. ncbi request reprint Plasma adiponectin levels in newborns are higher than those in adults and positively correlated with birth weight
    Yumiko Kotani
    Department of Pediatrics, School of Medicine, University of Tokushima, 3 Kuramoto cho, Japan
    Clin Endocrinol (Oxf) 61:418-23. 2004
    ..The aim of this study was to examine plasma adiponectin concentrations during perinatal the period and their correlations with fetal anthropometric parameters and other hormones...
  5. doi request reprint Successful allogeneic unrelated bone marrow transplantation using reduced-intensity conditioning for the treatment of X-linked adrenoleukodystrophy in a one-yr-old boy
    Kazumi Okamura
    Department of Pediatrics, University of Tokushima Graduate School of Medical Science, Tokushima, Japan
    Pediatr Transplant 13:130-3. 2009
    ..RIST should be considered as a pretransplant conditioning for ALD...
  6. ncbi request reprint Comparative clinico-pathological study of saposin-A-deficient (SAP-A-/-) and Twitcher mice
    Takashi Yagi
    Department of Pathology and Laboratory Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599 7525, USA
    J Neuropathol Exp Neurol 63:721-34. 2004
    ..These results suggest that peripheral macrophages are readily accessible in these sites and participate in the demyelinating process in the central nervous system...
  7. ncbi request reprint Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice
    Keiko Tadano-Aritomi
    Department of Biochemistry, Teikyo University School of Medicine, Kaga 2 11 1, Itabashi ku, Tokyo 173 8605, Japan
    J Lipid Res 44:1737-43. 2003
    ..These analytical findings on the two saposin mutants may shed some light on the physiological function of seminolipid and GalEAG...
  8. ncbi request reprint Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking
    Ying Sun
    Division of Human Genetics, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Hum Mol Genet 16:957-71. 2007
    ..This CD null mouse model provides a tool to explore the in vivo functional interactions of saposins in GSL metabolism and lysosomal storage diseases, and prosaposin's physiological effects...
  9. ncbi request reprint Hematopoietic cell transplantation ameliorates clinical phenotype and progression of the CNS pathology in the mouse model of late onset Krabbe disease
    Takashi Yagi
    Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA
    J Neuropathol Exp Neurol 64:565-75. 2005
    ..In contrast, the peripheral nerves showed features of hypertrophic neuropathy with hypomyelination and onion bulb formation, suggesting that there are different cellular responses to the BMT in the CNS and PNS...
  10. ncbi request reprint The function of sphingolipids in the nervous system: lessons learnt from mouse models of specific sphingolipid activator protein deficiencies
    Junko Matsuda
    Institute of Glycotechnology, Future Science and Technology Joint Research Center, Tokai University, Hiratsuka, Japan
    J Neurochem 103:32-8. 2007
    ..Collectively, the information provides support for the potential importance of sphingolipids in the function of the nervous system...