Neural Crest Cells are a transient embryonal population originating from the neural ectoderm border. NCCs migrate and generate differentiated cell types, including neurons and glia of the peripheral nervous systems, chromaffin cells, melanocytes, skeletal and connective components of the head. Alteration of the NCCs function causes developmental and congenital defects. Human NCCs are difficult to obtain and current knowledge derives mainly from animal models. Recently, human cell models have been obtained by embryonic stem cell or induced pluripotent stem cells but, up to date, in vitro data originate from engineered and/or tumoral cell lines.
In this study we developed a new human neural crest-derived cell model expressing neural crest specifier genes. NCC differentiated towards lineages originated in vivo by NCCs, induced a mature phenotype in a neuronal model acting like Schwann precursors, possessed migration activity and acquired a sympatho-adrenal phenotype.
In conclusion, this unique cell model provides a potential tool for studying the physiopathology of human fetal development and tumorigenesis. The effects caused on metabolism, functions and genetic profile of these cells by silencing pheochromocytoma/paraganglioma susceptibility genes, especially SDHB, may provide additional information on the pathogenesis of these tumors.