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Tumour aggressiveness in people with recessive dystrophic epidermolysis bullosa and the role of a short RNA molecule

New scientific publication from the EB House

Squamous cell carcinomas (SCC) are the most common cause of death in adults with recessive dystrophic EB (RDEB). However, these tumors do not always behave the same way. Some spread rapidly, while others remain locally confined for an extended period. Currently, there are limited treatment options for SCC in RDEB patients. Therefore, it is crucial to understand the mechanisms influencing tumor growth and spread. This understanding is vital for developing therapies and helping doctors to select the optimal therapy for their patients.  

Researchers at the EB House conducted a study to investigate the varied behavior of RDEB SCC. They examined both the appearance of cancer cells and specific molecular markers in the cells. They were particularly interested in understanding how the behavior of RDEB skin cancer cells changes, focusing on a process known as epithelial-mesenchymal transition (EMT). EMT describes the transition of cells from a tightly connected and organized state (epithelial) to a state with more flexible and mobile properties (mesenchymal). In cancer development, EMT can make cells more aggressive, enabling them to invade surrounding tissues more easily, and spread throughout the body (that is, forming metastases).

The study revealed that some RDEB SCC cells resembled normal skin cells, while others showed more aggressive characteristics. All examined cells exhibited both epithelial and mesenchymal markers, but in varying amounts, suggesting that RDEB tumors have different EMT states. These findings matched with the aggressiveness of the cancer and speed of disease progression in patients.

The researchers were further interested in identifying a microRNA that plays a role in EMT in RDEB tumors. MicroRNAs regulate genes and have thus come into focus as possible therapeutic molecules. They found that the concentration of MicroRNA-200b (miR-200b), a known regulator of EMT, was reduced in RDEB SCC. The lower miR-200b levels correlated with the more aggressive features in the tumors. Adding miR-200b to these cancer cells in a petri dish reduced their aggressive properties, causing the cells to behave more like normal skin cells.

In summary, this study illustrates that tumors in RDEB can exhibit different behaviors, especially in terms of aggressiveness. The application of miR-200b could serve as a promising approach to treat these aggressive cancer types.

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