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U.S. Department of Health and Human Services

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Thromb Haemost 2010 Sep;104(3):531-5

A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues.

Shomron N, Hamasaki-Katagiri N, Hunt R, Hershko K, Pommier E, Geetha S, Blaisdell A, Marple A, Roma I, Newell J, Allen C, Friedman S, Kimchi-Sarfaty C


Although ADAMTS13, the von Willebrand factor (VWF)-cleaving protease, is expressed in a range of tissues, the physiological significance of tissue-specific ADAMTS13 alternative splicing isoforms have yet to be clarified. Screening a panel of human tissues and cell lines revealed a spliced ADAMTS13 transcript in hepatic stellate cells and a hepatoma cell line that retains the 25th intron. A nonsense codon within the intron truncates the protease, which gains 64 novel amino acids in lieu of both CUB domains. This isoform, while retaining VWF-cleaving capability, accumulates intracellularly and its biological inaccessibility may prevent its participation in regulating haemostasis and other physiologic functions.

Category: Journal Article
PubMed ID: #20664912 DOI: 10.1160/TH09-12-0860
Includes FDA Authors from Scientific Area(s): Biologics
Entry Created: 2011-10-04 Entry Last Modified: 2012-08-29