A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues
| Autor: | Noam, Shomron, Nobuko, Hamasaki-Katagiri, Ryan, Hunt, Klilah, Hershko, Elie, Pommier, S, Geetha, Adam, Blaisdell, Alexandra, Dobkin, Andrew, Marple, Isabella, Roma, Jordan, Newell, Courtni, Allen, Scott, Friedman, Chava, Kimchi-Sarfaty |
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| Rok vydání: | 2009 |
| Předmět: |
Gene isoform
Pathology medicine.medical_specialty Carcinoma Hepatocellular medicine.medical_treatment Nonsense mutation ADAMTS13 Protein CHO Cells Transfection Gene Expression Regulation Enzymologic Article Substrate Specificity Cricetulus hemic and lymphatic diseases Cell Line Tumor Cricetinae von Willebrand Factor medicine Hepatic Stellate Cells Animals Humans Protein Isoforms RNA Messenger Protease business.industry Alternative splicing Liver Neoplasms Intron Hematology CUB domain Introns Cell biology Gene Expression Regulation Neoplastic ADAM Proteins Alternative Splicing Codon Nonsense Hepatic stellate cell business |
| Zdroj: | Thrombosis and haemostasis. 104(3) |
| ISSN: | 2567-689X |
| Popis: | SummaryAlthough 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. |
| Databáze: | OpenAIRE |
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