Satoshi Kono

Summary

Affiliation: Hamamatsu University School of Medicine
Country: Japan

Publications

  1. ncbi request reprint Aceruloplasminemia: an update
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan Electronic address
    Int Rev Neurobiol 110:125-51. 2013
  2. ncbi request reprint Aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Curr Drug Targets 13:1190-9. 2012
  3. doi request reprint Biological effects of mutant ceruloplasmin on hepcidin-mediated internalization of ferroportin
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Biochim Biophys Acta 1802:968-75. 2010
  4. ncbi request reprint Dominant-negative effects of a novel mutation in the filamin myopathy
    S Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Neurology 75:547-54. 2010
  5. doi request reprint Functional brain imaging in glucocerebrosidase mutation carriers with and without parkinsonism
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Handayama, Hamamatsu, Japan
    Mov Disord 25:1823-9. 2010
  6. ncbi request reprint Cys-881 is essential for the trafficking and secretion of truncated mutant ceruloplasmin in aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    J Hepatol 47:844-50. 2007
  7. ncbi request reprint Dopaminergic neuronal dysfunction associated with parkinsonism in both a Gaucher disease patient and a carrier
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    J Neurol Sci 252:181-4. 2007
  8. ncbi request reprint Molecular and pathological basis of aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Biol Res 39:15-23. 2006
  9. ncbi request reprint Biochemical features of ceruloplasmin gene mutations linked to aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Neuromolecular Med 8:361-74. 2006
  10. ncbi request reprint Hepatic iron overload associated with a decreased serum ceruloplasmin level in a novel clinical type of aceruloplasminemia
    Satoshi Kono
    The First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Gastroenterology 131:240-5. 2006

Collaborators

Detail Information

Publications25

  1. ncbi request reprint Aceruloplasminemia: an update
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan Electronic address
    Int Rev Neurobiol 110:125-51. 2013
    ..The aim of this chapter is to provide an overview of not only the clinical features, genetic and molecular pathogenesis, and treatment of aceruloplasminemia but also the biological and physiological features of iron metabolism. ..
  2. ncbi request reprint Aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Curr Drug Targets 13:1190-9. 2012
    ....
  3. doi request reprint Biological effects of mutant ceruloplasmin on hepcidin-mediated internalization of ferroportin
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Biochim Biophys Acta 1802:968-75. 2010
    ..The properties of mutant ceruloplasmin regarding the regulation of ferroportin may therefore provide a therapeutic strategy for aceruloplasminemia patients...
  4. ncbi request reprint Dominant-negative effects of a novel mutation in the filamin myopathy
    S Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Neurology 75:547-54. 2010
    ..Filamin myopathy is associated with mutations in the filamin C gene (FLNC) and is a myofibrillar myopathy characterized by focal myofibrillar destruction and cytoplasmic aggregates containing several Z-disk-related proteins...
  5. doi request reprint Functional brain imaging in glucocerebrosidase mutation carriers with and without parkinsonism
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, Handayama, Hamamatsu, Japan
    Mov Disord 25:1823-9. 2010
    ..The hypometabolism in the SMA may, therefore, be involved in the clinical characteristics of parkinsonism associated with GBA mutations when the carriers manifest parkinsonism...
  6. ncbi request reprint Cys-881 is essential for the trafficking and secretion of truncated mutant ceruloplasmin in aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    J Hepatol 47:844-50. 2007
    ..The aim of this study was to elucidate the molecular pathogenesis of aceruloplasminemia by a functional analysis of mutant ceruloplasmin...
  7. ncbi request reprint Dopaminergic neuronal dysfunction associated with parkinsonism in both a Gaucher disease patient and a carrier
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    J Neurol Sci 252:181-4. 2007
    ....
  8. ncbi request reprint Molecular and pathological basis of aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Biol Res 39:15-23. 2006
    ..The investigation of mutant ceruloplasmin reveals new insights into molecular pathogenesis of aceruloplasminemia as well as biosynthesis, trafficking, and function of ceruloplasmin...
  9. ncbi request reprint Biochemical features of ceruloplasmin gene mutations linked to aceruloplasminemia
    Satoshi Kono
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Neuromolecular Med 8:361-74. 2006
    ..Therefore, the mutant protein was considered to have accumulated in the ER thus leading to the ER stress, which resulted in cell death...
  10. ncbi request reprint Hepatic iron overload associated with a decreased serum ceruloplasmin level in a novel clinical type of aceruloplasminemia
    Satoshi Kono
    The First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Gastroenterology 131:240-5. 2006
    ..Aceruloplasminemia is a novel hereditary iron overload disease caused by a mutation in the ceruloplasmin gene and characterized by a complete deficiency of serum ceruloplasmin and iron accumulation in the liver and brain...
  11. doi request reprint SPG3A-linked hereditary spastic paraplegia associated with cerebral glucose hypometabolism
    Tatsuhiro Terada
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Ann Nucl Med 27:303-8. 2013
    ....
  12. doi request reprint Benign hereditary chorea: dopaminergic brain imaging in patients with a novel intronic NKX2.1 gene mutation
    Takashi Konishi
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1, Handayama, Hamamatsu, 431 3192, Japan
    J Neurol 260:207-13. 2013
    ..The molecular brain imaging and therapeutic response may help elucidate the pathophysiological mechanism of the motor control in the BHC-associated NKX2.1 mutation...
  13. ncbi request reprint Aceruloplasminemia, an inherited disorder of iron metabolism
    Hiroaki Miyajima
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Biometals 16:205-13. 2003
    ..These findings suggest that iron-mediated free radicals causes neuronal cell damage through lipid peroxidation and mitochondrial dysfunction in aceruloplasminemia brains...
  14. ncbi request reprint Glucose and oxygen hypometabolism in aceruloplasminemia brains
    Hiroaki Miyajima
    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu
    Intern Med 41:186-90. 2002
    ..To clarify the free radical-mediated tissue injury caused by intracellular iron accumulation through mitochondrial dysfunction...
  15. ncbi request reprint Increased lipid peroxidation and mitochondrial dysfunction in aceruloplasminemia brains
    Hiroaki Miyajima
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1 Handayama, Hamamatsu 431 3192, Japan
    Blood Cells Mol Dis 29:433-8. 2002
    ..These findings suggest that iron-mediated free radicals may contribute to neuronal cell damage through increased lipid peroxidation and the impairment of mitochondrial energy metabolism in aceruloplasminemia brains...
  16. ncbi request reprint GABA-A receptor impairment in cerebellar ataxia with anti-glutamic acid decarboxylase antibodies
    Yasushi Hosoi
    First Department of Medicine, Hamamatsu University School of Medicine, 1 20 1, Handayama, Hamamatsu, 431 3192, Japan
    J Neurol 260:3086-92. 2013
    ....
  17. pmc In vivo mesolimbic D2/3 receptor binding predicts posttherapeutic clinical responses in restless legs syndrome: a positron emission tomography study
    Yumi Oboshi
    Department of Biofunctional Imaging, Medical Photonics Research Center, Hamamatsu University School of Medicine, Hamamatsu, Japan
    J Cereb Blood Flow Metab 32:654-62. 2012
    ..The present results suggest that alterations in mesolimbic D2/3 receptor function reflect the pathophysiology of iRLS, and the baseline availability of D2/3 receptors may predict the clinical outcome after D2/3 agonist treatment...
  18. doi request reprint A case of Epstein-Barr virus associated post-transplant lymphoproliferative disorder with CNS involvement: pathological findings at both biopsy and autopsy
    Makiko Suzuki
    Departments of Neurology and Pathology, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Neuropathology 31:440-5. 2011
    ..We herein describe the relationship between clinical and pathological findings and demonstrate the way CNS PTLD lesion progresses...
  19. ncbi request reprint An autopsy case of the Marburg variant of multiple sclerosis (acute multiple sclerosis)
    Makiko Suzuki
    The First Department of Medicine, Department of Neurology, Hamamatsu University School of Medicine, Japan
    Intern Med 52:1825-32. 2013
    ..We evaluate the pathogenic differences between the Marburg type and prototypical MS by discussing the neuropathology and cerebrospinal fluid (CSF) findings of our case. ..
  20. ncbi request reprint [Familial Wernicke's-like encephalopathy]
    Hiroaki Miyajima
    First Department of Medicine, Hamamatsu University School of Medicine
    Rinsho Shinkeigaku 50:855-7. 2010
    ..High expression of SLC19A3 RNA in the thalamus may explain the selective thalamic lesions on MRI. The identification of this syndrome proves insight into the thiamine metabolism associated with Wernicke's encephalopathy in humans...
  21. doi request reprint Brain and liver iron accumulation in aceruloplasminemia
    Koji Fujita
    From the Department of Clinical Neuroscience K F, Y O, Y I, R K and Radiology M H, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima, Japan and the First Department of Medicine S K, H M, Hamamatsu University School of Medicine, Hamamatsu, Japan
    Neurology 81:2145-6. 2013
    ..W858X) in the ceruloplasmin gene, confirming the diagnosis of aceruloplasminemia.(1) This case highlights the importance of T2*-weighted MRI in diagnosing aceruloplasminemia, even in patients with minimal neurologic symptoms. ..
  22. ncbi request reprint Frontal lobe dysfunction associated with glucose hypometabolism in aceruloplasminemia
    Hiroaki Miyajima
    J Neurol 252:996-7. 2005
  23. ncbi request reprint Mechanisms of copper incorporation into human ceruloplasmin
    Nathan E Hellman
    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 277:46632-8. 2002
    ....
  24. ncbi request reprint Biochemical analysis of a missense mutation in aceruloplasminemia
    Nathan E Hellman
    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Biol Chem 277:1375-80. 2002
    ..Taken together, these studies reveal new insights into the determinants of holoceruloplasmin biosynthesis and indicate that aceruloplasminemia can result from retention of mutant ceruloplasmin within the early secretory pathway...
  25. ncbi request reprint Autosynchronized systolic unloading during left ventricular assist with a centrifugal pump
    Satoshi Kono
    Department of Cardiovascular Surgery, Graduate School of Medicine, Kyoto University, Japan
    J Thorac Cardiovasc Surg 125:353-60. 2003
    ..The purpose of this study was to investigate how the inflow cannulation site of the left ventricular assist system with a centrifugal pump would influence cardiac function on failing heart models...