Abstract:
: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 alpha-smooth muscle actin, confirming that these cells were activated HSCs. These results suggest that HSCs may be major cells producing ADAMTS13 in human liver.
journal_name
Bloodjournal_title
Bloodauthors
Uemura M,Tatsumi K,Matsumoto M,Fujimoto M,Matsuyama T,Ishikawa M,Iwamoto TA,Mori T,Wanaka A,Fukui H,Fujimura Ydoi
10.1182/blood-2005-01-0152subject
Has Abstractpub_date
2005-08-01 00:00:00pages
922-4issue
3eissn
0006-4971issn
1528-0020pii
2005-01-0152journal_volume
106pub_type
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