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Koichi Kokame. Department of Molecular Pathogenesis, National Cerebral and Cardiovascular Center, Osaka, Japan. Koichi Kokame was born in 1967 in Hiroshima (Japan), grew up in Hiroshima, Fukuoka, and Kyoto, and now lives in Osaka. He entered Faculty of Science, Kyoto University in 1985. Then, he went on to Department of Biophysics, Graduate School of Science, Kyoto University in 1989, and undertook a basic study on the light signal transduction in photoreceptor cells. He finished a doctorate of Science at Kyoto University in 1994. His thesis was based on a paper entitled “Lipid modification at the N terminus of photoreceptor G-protein alpha-subunit” (Nature 359, 749). In 1994, he joined Department of Molecular Pathogenesis, National Cerebral and Cardiovascular Center in Osaka as a staff scientist, and shifted research subjects from photoreceptor cells to hemostasis and thrombosis.

One of his current research subjects is endoplasmic reticulum (ER)-associated protein degradation (ERAD). In 1996, he published a paper entitled “Homocysteine-respondent genes in vascular endothelial cells identified by differential display analysis. GRP78/BiP and novel genes” (JBC 271, 29659). One of the novel genes was HERPUD1, which encodes an ER-membrane protein Herp. Herp expression is strongly induced by ER stress, and Herp is one of the key proteins in ERAD.

The other subjects of his research are plasma proteins. In 2002, he published a paper entitled “Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity” (PNAS 99, 11902). The functional deficiency of ADAMTS13 (metalloprotease) leads to thrombotic thrombocytopenic purpura (TTP), and that of von Willebrand factor (VWF, a substrate of ADAMTS13) leads to von Willebrand disease. His main impact has been made in the applicability of ADAMTS13 activity assay, which is widely used for the diagnosis of TTP.

He became a principal investigator (Director, Department of Molecular Pathogenesis) in 2015, and the laboratory members are studying ERAD, ADAMTS13, VWF, and so on.