Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer

Neuroblastoma is a common extracranial solid tumour of childhood, responsible for 15% of cancer-related deaths in children. Prognoses vary from spontaneous remission to aggressive disease with extensive metastases, where treatment is challenging. Tumours are thought to arise from sympathoadrenal pro...

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Autores principales:	Anna M. Wulf, Marcela M. Moreno, Chloé Paka, Alexandra Rampasekova, Karen J. Liu
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	neuroblastoma anaplastic lymphoma kinase ALK neural crest Biology (General) QH301-705.5 Chemistry QD1-999
Acceso en línea:	https://doaj.org/article/30c5123817bf4abb973de14d69d67827
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spelling	oai:doaj.org-article:30c5123817bf4abb973de14d69d678272021-11-11T17:10:32ZDefining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer10.3390/ijms2221117181422-00671661-6596https://doaj.org/article/30c5123817bf4abb973de14d69d678272021-10-01T00:00:00Zhttps://www.mdpi.com/1422-0067/22/21/11718https://doaj.org/toc/1661-6596https://doaj.org/toc/1422-0067Neuroblastoma is a common extracranial solid tumour of childhood, responsible for 15% of cancer-related deaths in children. Prognoses vary from spontaneous remission to aggressive disease with extensive metastases, where treatment is challenging. Tumours are thought to arise from sympathoadrenal progenitor cells, which derive from an embryonic cell population called neural crest cells that give rise to diverse cell types, such as facial bone and cartilage, pigmented cells, and neurons. Tumours are found associated with mature derivatives of neural crest, such as the adrenal medulla or paraspinal ganglia. Sympathoadrenal progenitor cells express <i>anaplastic lymphoma kinase (ALK)</i>, which encodes a tyrosine kinase receptor that is the most frequently mutated gene in neuroblastoma. Activating mutations in the kinase domain are common in both sporadic and familial cases. The oncogenic role of ALK has been extensively studied, but little is known about its physiological role. Recent studies have implicated ALK in neural crest migration and sympathetic neurogenesis. However, very few downstream targets of ALK have been identified. Here, we describe pathological activation of ALK in the neural crest, which promotes proliferation and migration, while preventing differentiation, thus inducing the onset of neuroblastoma. Understanding the effects of ALK activity on neural crest cells will help find new targets for neuroblastoma treatment.Anna M. WulfMarcela M. MorenoChloé PakaAlexandra RampasekovaKaren J. LiuMDPI AGarticleneuroblastomaanaplastic lymphoma kinaseALKneural crestBiology (General)QH301-705.5ChemistryQD1-999ENInternational Journal of Molecular Sciences, Vol 22, Iss 11718, p 11718 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	neuroblastoma anaplastic lymphoma kinase ALK neural crest Biology (General) QH301-705.5 Chemistry QD1-999
spellingShingle	neuroblastoma anaplastic lymphoma kinase ALK neural crest Biology (General) QH301-705.5 Chemistry QD1-999 Anna M. Wulf Marcela M. Moreno Chloé Paka Alexandra Rampasekova Karen J. Liu Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
description	Neuroblastoma is a common extracranial solid tumour of childhood, responsible for 15% of cancer-related deaths in children. Prognoses vary from spontaneous remission to aggressive disease with extensive metastases, where treatment is challenging. Tumours are thought to arise from sympathoadrenal progenitor cells, which derive from an embryonic cell population called neural crest cells that give rise to diverse cell types, such as facial bone and cartilage, pigmented cells, and neurons. Tumours are found associated with mature derivatives of neural crest, such as the adrenal medulla or paraspinal ganglia. Sympathoadrenal progenitor cells express <i>anaplastic lymphoma kinase (ALK)</i>, which encodes a tyrosine kinase receptor that is the most frequently mutated gene in neuroblastoma. Activating mutations in the kinase domain are common in both sporadic and familial cases. The oncogenic role of ALK has been extensively studied, but little is known about its physiological role. Recent studies have implicated ALK in neural crest migration and sympathetic neurogenesis. However, very few downstream targets of ALK have been identified. Here, we describe pathological activation of ALK in the neural crest, which promotes proliferation and migration, while preventing differentiation, thus inducing the onset of neuroblastoma. Understanding the effects of ALK activity on neural crest cells will help find new targets for neuroblastoma treatment.
format	article
author	Anna M. Wulf Marcela M. Moreno Chloé Paka Alexandra Rampasekova Karen J. Liu
author_facet	Anna M. Wulf Marcela M. Moreno Chloé Paka Alexandra Rampasekova Karen J. Liu
author_sort	Anna M. Wulf
title	Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
title_short	Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
title_full	Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
title_fullStr	Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
title_full_unstemmed	Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer
title_sort	defining pathological activities of alk in neuroblastoma, a neural crest-derived cancer
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/30c5123817bf4abb973de14d69d67827
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Defining Pathological Activities of ALK in Neuroblastoma, a Neural Crest-Derived Cancer

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