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Blood, 1 August 2005, Vol. 106, No. 3, pp. 922-924.
Prepublished online as a Blood First Edition Paper on April 26, 2005; DOI 10.1182/blood-2005-01-0152.
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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Brief report
Localization of ADAMTS13 to the stellate cells of human liver
Masahito Uemura,
Kouko Tatsumi,
Masanori Matsumoto,
Masao Fujimoto,
Tomomi Matsuyama,
Masatoshi Ishikawa,
Taka-aki Iwamoto,
Toshio Mori,
Akio Wanaka,
Hiroshi Fukui, and
Yoshihiro Fujimura
From the Third Department of Internal Medicine, the Departments of Anatomy and Blood Transfusion Medicine, and the Radioisotope Research Center, Nara Medical University, Kashihara, Nara, Japan.
Although the chromosomal localization (9q34) of the gene encoding the human form of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13) and its exclusive expression in the liver have been established, the cells that produce this enzyme are yet to be determined. We investigated the expression of ADAMTS13 mRNA and protein in fresh frozen specimens obtained during liver biopsies of 8 patients with liver diseases. In situ hybridizations to localize ADAMTS13 mRNA showed positive signals exclusively in perisinusoidal cells with irregularly elongated dendritic processes extending between hepatocytes. Furthermore, ADAMTS13 was detected immunohistochemically in perisinusoidal cells, whereas no staining was observed in hepatocytes. The positive cells varied in shape from unipolar to dendritic with irregularly elongated cytoplasmic processes, features common to hepatic stellate cells (HSCs). Double-labeling experiments revealed that the ADAMTS13-positive cells also expressed  -smooth muscle actin, confirming that these cells were activated HSCs. These results suggest that HSCs may be major cells producing ADAMTS13 in human liver.
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