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ALK pERKs up MYCN in neuroblastoma
Sven Lindner1, Anton Henssen2, Kathy Astrahantseff3
1Department of Pediatric Oncology and Hematology, University Children's Hospital Essen, 45122 Essen, Germany. German Cancer Consortium (DKTK), Partner Site Essen/Duesseldorf, 45122 Essen, Germany. Department of Translational Neuro-Oncology, West German Cancer Center, University Hospital Essen, University Duisburg-Essen, Hufelandstr.55, 45122 Essen, Germany. German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg, Germany. Centre for Medical Biotechnology, University Duisburg-Essen, 45122 Essen, Germany.
Abstract:
The gene expressing the receptor tyrosine kinase anaplastic lymphoma kinase (ALK) is mutated and aberrantly expressed in several cancers. The clinical efficacy of the ALK inhibitor, crizotinib, lags behind expectations for treating MYCN-amplified, ALK-mutant neuroblastoma, a deadly childhood cancer. In this issue of Science Signaling, Umapathy et al. identify the kinase extracellular signal-regulated kinase 5 (ERK5) as a central mediator that enables ALK to boost MYCN expression, and they show that inhibiting ERK5 in concert with ALK reduced neuroblastoma cell viability in vitro and in xenograft tumor models. This report has important clinical implications for the treatment of patients with neuroblastoma or other tumors that overexpress MYC(N) and harbor ALK mutations, such as non-small-cell lung cancer.
Insights
Anaplastic lymphoma kinase (ALK) drives neuroblastoma by boosting MYCN expression via extracellular signal-regulated kinase 5 (ERK5). Inhibiting both ALK and ERK5 shows promise for treating this deadly childhood cancer.
Area of Science:
- Oncology
- Molecular Biology
- Cancer Genetics
Background:
- Anaplastic lymphoma kinase (ALK) mutations are implicated in various cancers.
- Crizotinib, an ALK inhibitor, has limited efficacy in MYCN-amplified, ALK-mutant neuroblastoma.
- Neuroblastoma is a deadly childhood cancer with poor prognosis in certain genetic subtypes.
Purpose of the Study:
- To identify the molecular mechanisms by which ALK promotes neuroblastoma.
- To investigate the role of extracellular signal-regulated kinase 5 (ERK5) in ALK-driven neuroblastoma.
- To evaluate the therapeutic potential of combined ALK and ERK5 inhibition.
Main Methods:
- Investigated the interaction between ALK and MYCN signaling pathways.
- Utilized cell viability assays and xenograft tumor models.
- Assessed the effects of ALK and ERK5 inhibition on neuroblastoma cells.
Main Results:
- Extracellular signal-regulated kinase 5 (ERK5) acts as a key mediator for ALK to enhance MYCN expression.
- Combined inhibition of ALK and ERK5 significantly reduced neuroblastoma cell viability in vitro.
- Dual ALK and ERK5 inhibition demonstrated efficacy in preclinical xenograft tumor models.
Conclusions:
- ERK5 is a critical downstream effector of ALK in promoting neuroblastoma.
- Targeting both ALK and ERK5 simultaneously offers a promising therapeutic strategy for neuroblastoma.
- This approach may also benefit other cancers with MYC(N) overexpression and ALK mutations, such as non-small-cell lung cancer.
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