- Marusiak, Anna A;
- Edwards, Zoe C;
- Hugo, Willy;
- Trotter, Eleanor W;
- Girotti, Maria R;
- Stephenson, Natalie L;
- Kong, Xiangju;
- Gartside, Michael G;
- Fawdar, Shameem;
- Hudson, Andrew;
- Breitwieser, Wolfgang;
- Hayward, Nicholas K;
- Marais, Richard;
- Lo, Roger S;
- Brognard, John
RAF inhibitor therapy yields significant reductions in tumour burden in the majority of V600E-positive melanoma patients; however, resistance occurs within 2-18 months. Here we demonstrate that the mixed lineage kinases (MLK1-4) are MEK kinases that reactivate the MEK/ERK pathway in the presence of RAF inhibitors. Expression of MLK1-4 mediates resistance to RAF inhibitors and promotes survival in V600E-positive melanoma cell lines. Furthermore, we observe upregulation of the MLKs in 9 of 21 melanoma patients with acquired drug resistance. Consistent with this observation, MLKs promote resistance to RAF inhibitors in mouse models and contribute to acquired resistance in a cell line model. Lastly, we observe that a majority of MLK1 mutations identified in patients are gain-of-function mutations. In summary, our data demonstrate a role for MLKs as direct activators of the MEK/ERK pathway with implications for melanomagenesis and resistance to RAF inhibitors.