Principal Investigator/Program Director (Last, First, Middle):

BIOGRAPHICAL SKETCH
Provide the following information for the key personnel and other significant contributors in the order listed on Form Page 2.
Follow this format for each person. DO NOT EXCEED FOUR PAGES.
NAMEPOSITION TITLE

Hiroko Kodama

Professor,
Department of Pediatrics, Teikyo University Schoolof Medicine, Tokyo, JapaneRA COMMONS USER NAMEoa

EDUCATION/TRAINING (Begin with baccalaureate or other initial professional education, such as nursing, and include postdoctoral training.)
INSTITUTION AND LOCATION

Osaka University, Osaka, Japan
Osaka University, Osaka, Japan

DEGREE(if applicable)

YEAR(s)Dr.Med.
PhD

1970
1982

FIELD OF STUDY

Medicine
Medicine

A. Positions and Honors.
Positions and Employment
1975-1982 Research Associate, Department of Pediatrics Osaka University, Osaka Japan
1982-1988 Assistant Professor, Department of Pediatrics, Jichi Medical University School of Medicine, Tochigi, Japan
1988-1990 Assistant Professor, Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan
1990-2005 Associate Professor, Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan
1991-2004 Past-time instructor, Department of Pediatrics Osaka University, Osaka Japan
2001-present Past-time instructor, Department of Developmental medicine, Tokyo University, Tokyo Japan
2005-present Professor, Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan
Other Experience and Professional Memberships
2007-present Vice predsident, International Society for Trace Element Research in Humans
2004-2007 Secretary, International Society for Trace Element Research in Humans
2002-present Director, Japan Society for Biomedical Research on Trace Elements
2007-present Director , Japanese Society for Inherited Metabolic Diseases
2003-present Councilor, The Japan Endocrine Society
2003-present Councilor, The Japanese Society of Clinical Nutrition
2003-present Organizer, Japanese Society for Child Nutrition Research
1998-present Councilor, Japanese Society for Pediatric Gastroenterology, Hepatology and Nutrition
1995-present Councilor, The Japanese Society of Child Neurology
1994-present Councilor, Japanese Society for Inherited Metabolic Diseases
2000-present Councilor, The Japanese Society for Pediatric Endocrinology
Present
The Society for the Study of Inborn Errors of Metabolism, Member
International Child Neurology Association, Member
Japan Pediatric Society, Member
The Japanese Biochemical Society, Member
The Japanese Society for Gene Diagnosis and Therapy, Member
Japan Neuroendocrine Society, Member
International Society for Trace Element Research in Humans, Secretary
The Japan Endocrine Society, Councilor
The Japanese Society of Clinical Nutrition, Councilor
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Principal Investigator/Program Director (Last, First, Middle):

Japanese Society for Child Nutrition Research, Organizer
Japan Society for Biomedical Research on Trace Elements, Director
Japanese Society for Pediatric Gastroenterology, Hepatology and Nutrition, Councilor
The Japanese Society of Child Neurology, Councilor
Japanese Society for Inherited Metabolic Diseases, Councilor
The Japanese Society for Pediatric Endocrinology, Councilor
Meeting President
13th Annual Meeting of Japanese Society for Pediatric Gastroenterology, Hepatology and Nutrition 2004
12th Annual Meeting of Japanese Society for Child Growth 2004
45th Annual Meeting of Japanese Society of Child Neurology in Kanto 2006
19th Annual Meeting of Japan Society for Biomedical Research on Trace Elements 2008
51st Annual Meeting of the Japanese Society for Inherited Metabolic Disease·8th Asian Symposium for InheritedMetabolic Diseases 200919th Annual Meeting of Society for LEC rat 2009
6th Annual Meeting of Japanese Society for Child Nutrition Research 2009

B. Selected peer reviewed publications (from 2001)
1. Kodama H, Fujisawa C Copper Metabolism and inherited copper transport disorders: molecular mechanisms,
screening, and treatment Metallomics 1:42-52 2009
2. Hikita T, Kodama H, Nakamoto N, Kaga F, Amakata K, Ogita K, Kaneko S, Fujii Y, YanagawaY Effective
Prophylactic therapy for cyclic vomiting syndrome in children using valproate Brain Dev. 31:411-413, 2009
3. Uegaki S,Tanaka A,Mori Y,Kodama H,Fukusato T,Takikawa H Successful treatment with colestimide for a boutof cholestasis in a Japanese patient with benign recurrent intrahepatic cholestasis caused by ATP8B1 mutation
Intem Med 47(7):599-602, 2008
4. Kodama H, Fujisawa F, Gu YH, Shiga K. Copper metabolism and copper transport disorders. Biomed Res TraceElements 18: 249-254, 2007
5. Nakagawa T, Inoue Y, Kodama H, Yamazaki H, Kawai K, Suemizu H, Masuda R, Iwazaki M, Ymamada S,Ueyama Y, Inoue H, Nakamura M. Expression of copper-transporting P-type adenosine triphosphatase(ATP7B)
correlates with cisplatin resistance in human non-small cell lung cancer xenografts. Oncol Rep. 20(2):265-70. 2008
6. Gu YH, Gu QJ, Xu SF, Sun HJ, Du SL, Kodama H. Genetic Variation in ATP7B Promoter and 5' UTR in Han
Chinese Patients with Wilson's Disease. Biomed Res Trace Elements 18(3): 264-268, 2007
7. Kobayashi K, Katsuya Y, Abdulah R, Fujisawa C, Nagamine T, Morikawa A, Murakami M, Koda Kodama H.
Direct Analysis of Ceruloplasmin in Human Blood Serum by HPLC/Inductively Coupled Plasma-Mass
Spectrometry for the Diagnosis of Wilson Disease. Biomed Res Trace Elements 18(1): 91-95, 2007
8. Gu YH, Kodama H, Watanabe S, Kikuchi N, Ishitsuka I, Ozawa H, Fujisawa C, Shiga K. The first reported case ofMenkes disease caused by an Alu insertion mutation. Brain Dev 29: 105-108, 2007
9. Kodama H, Sato E, Gu YH, Shiga K, Fujisawa C, Kozuma T. Effect of copper and diethyldithyldithiocarrbamate
combination therapy on the macular mouse, an animal model of Menkes disease J Inherit Metab Dis 28:971-978,2005
10. Gu YH, Kodama H, Du SL. Apolipoprotein E genotype analysis in Chinese Han ethnic children with Wilson'sdisease, with a concentration on those homozygous for R778L. Brain Dev 27(8):551-553. 2005
11. Kobayashi S, Kodama H, Inuzuka R, Mori Y, Yanagawa Y. Combination treatment with penicillamine andtrientine in a patient with Wilson's disease. Pediatr Int. 47(5): 589-591, 2005
12. Matsuo M, Tasaki R, Kodama H, Hamasaki Y. Screening for Menkes disease using the urine HVA/VMA ratio. JInherit Metab Dis 28(1): 89-93, 2005
13. Gu YH, Kodama H, Shiga K, Nakata S, Yanagawa Y, Ozawa H. A survey of Japanese patients with Menkes
disease from 1990 to 2003: incidence and early signs before typical symptomatic onset, pointing the way to earlier
diagnosis. J Inherit Metab Dis 28(4): 473-478, 2005
14. Kako K, Takehara A, Arai H, Onodera T, Takahashi Y, Hanagata H, Ogra Y, Takagi H, Kodama H, Suzuki KT,
Munekata E, Fukamizu A. A selective requirement for copper-dependent activation of cytochrome c oxidase byCox17p. Biochem Biophys Res Commun 324:1379-1385, 2004
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Principal Investigator/Program Director (Last, First, Middle):

15. Sasaki G, Ishii T, Sato S, Hoshino K, Morikawa Y, Kodama H, Matsuo N, Takahashi T, Hasegawa T. Multiple
polypoid masses in the gastrointestinal tract in patient with Menkes disease on copper-histidinate therapy. Eur J
Pediatr 163(12): 745-6, 2004
16. Gu YH, Shiga K, Kodama H, Du SL, Shimizu N, Takeshita Y, Aoki T. Genotype-phenotype analysis of mutation
R778L in the ATP7B gene. Biomed Res Trace Elements 15(1): 33-36 2004
17. Gu YH, Kodama H, Du SL, Gu QJ, Sun HJ, Ushijima H. Mutation spectrum and polymorphisms in ATP7B
identified on direct sequencing of all exons in Chinese Han and Hui ethnic patients with Wilson's disease. Clin
Genet 64: 479-484, 2003
18. Fu X, Rinaldo P, Hahn SH, Kodama H, Packman S. Mutation analysis of copper transporter genes in patients with
ethylmalonic encephalopathy, mitochondriopathies and copper deficiency phenotypes. J Inherit. Metab Dis 26:55-66, 2003
19. Kodama H, Sato E, Yanagawa Y, Ozawa H, Kozuma T. Biochemical indicator for evaluation of connective tissueabnormalities in Menkes' disease. J Pediatr 142: 726-728, 2003
20. Ozawa H, Kodama H, Kawaguchi H, Mochizuki T, Kobayashi M, Igarashi T. Renal function in patients withMenkes disease. Eur J Pediatr 162: 51-52, 2003
21. Kodama H, Gu Y-H, Shiga K, Mori Y, Yanagawa Y, Mizunuma M, Sato E, Nakamoto N. Genotype and phenotypein patients with Menkes disease and occipital horn syndrome. J Inherit Metab Dis. 25: 166, 2002
22. Shiihara T, Kato M, Honma T, Kimura T, Matsunaga A, Kodama H, Hayasaka K. Progressive Slidinng Hiatal
Hernia as a Complication of Menkes' Syndrome. J Child Neurol 17: 401-402, 2002
23. Takahashi Y, Kako K, Kashiwabara S, Takehara A, Inada Y, Arai H, Nakada K, Kodama H, Hayashi J, Baba T,Munekata E. Mammalian copper chaperone cox17p has an essential role in activation of cytochrome c oxidase andembryonic development. Molecular and Cellular Biology 22(21): 7614-7621, 2002
24. Gu Y, Kodama H, Sato E, Mochizuki D, Yanagawa Y, Takayanagi M, Sato K, Ogawa A, Ushijima H, Lee CC.
Prenatal diagnosis of Menkes disease by genetic analysis and copper measurement. Brain Dev 24: 715-718, 2002
25. Murakami H, Kodama H, Nemoto N. Abnormality of vascular elastic fibers in the macular mouse and a patientwith Menkes'disease:ultrastructural and immunohistochemical study. Med Electron Microsc 35: 24-30, 2002
26. Komatsu H, Fujisawa T, Inui A, Sogo T, Sekine I, Kodama H, Uemoto S, Tanaka K. Hepatic copper concentrationin children undergoing living related liver transplantation due to Wilsonian fulminant hepatic failure. ClinicalTransplantation 16: 227-232, 2002
27. Du SL, Len T, Gu YH, Ushijima H, Kodama H. Long-term treatment with high-dose zinc sulphate in 36 Children
with Wilson's disease. Biomed Res Trace Elements 13: 85-88, 2002
28. Kodyama H, Gu YH, Mizunuma M. Drug targets in Menkes disease - prospective developments. Expert Opin TherTargets. 5(5):625-635, 2001
29. Ozawa H, Kodama H, Murata Y, Takashima S, Noma S. Transient temporal lobe changes and a novel mutation ina patient with Menkes disease. Pediatr Int. 43: 437-40, 2001
30. Aita K, Jin Y, Irie H, Takahashi I, Kobori K, Nakasato Y, Kodama H, Yanagawa Y, Yoshikawa T, Shiga J. Are
there histopathologic characteristics particular to fulminant hepatic failure caused by human herpesvirus-6
infection? A case report and discussion. Hum Pathol 32: 887-889, 2001
31. Tsai FJ, Lee CC, Wu MC, Lin SP, Lin CY, Tsai CH, Kodama H, Wu JY. Mutation analysis of type II Gaucher
disease in five Taiwanese children:identification of two novel mutations. Acta Paediatr Taiwan 42: 231-235, 2001
32. Gu YH, Koyama H, Murata Y, Mochizuki D, YanagawaY, Ushijima H, Shiba T, Lee CC. ATP7a gene mutations in
16 patients with Menkes disease and a patient with occipital horn syndrome. Am J Med Genet 99: 217-222, 2001
Books and chapters
1. Kodama H. (2002) Gene defects and clinical aspects in Menkes disease and occipital horn syndrome. Massaro E ed.
Handbook of copper pharmacology. Human Press. Totowa(USA). 319-338
2. Abe T, Araki K, Kobayashi M, Fujita Y, Shinozaki T, Kodama H, Ushijima H. (1998) Viral encephalitis : newconcept and treatment. Monduzzl rdifore new developments in child neurology. Monduzzl Rditore. Bologna(Italy).
341-348
3. Kodama H, Chang LM. Ed. (1996) Genetic disorders of copper metabolism. toxicology of metals. Boca Raton.
CRC Press Inc. 367-382
4. Kodama H, Nose O, Okada S, Yabuuchi H. (1983) The study of organic acids metabolism in a patient withornithine transcarbamylase (OTC) deficiency Lowenthal A, Mori A, Marescan B eds Urea Cycle Diseases. Plenum
Publishing Corporation. New York. 341-350
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