Generation of four induced pluripotent stem cell lines from a family harboring a single nucleotide variant in SCN5A

Patient-derived induced pluripotent stem cells (iPSC) are a valuable approach to model cardiovascular diseases. We nucleofected non-integrating episomal vectors in skin fibroblasts of three family members carrying a single nucleotide variant (SNV) in SCN5A, which encodes the cardiac-type sodium chan...

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Detalhes bibliográficos
Autores: Martinez Moreno, Rebecca, Carreras, David, Aran, Begoña, Kuebler, Bernd, Sarquella Brugada, Georgia, Brugada, Ramon, Pérez, Guillermo J., Scornik, Fabiana S., Selga, Elisabet
Formato: artículo
Fecha de publicación:2022
País:España
Recursos:UVic-UCC
Repositorio:RiUVic. Repositori institucional de la UVic-UCC
OAI Identifier:oai:dspace.uvic.cat:10854/8078
Acesso em linha:http://hdl.handle.net/10854/8078
https://doi.org/10.1016/j.scr.2022.102717
Access Level:acceso abierto
Palavra-chave:Sistema cardiovascular -- Malalties
Cor -- Malalties
Descrição
Resumo:Patient-derived induced pluripotent stem cells (iPSC) are a valuable approach to model cardiovascular diseases. We nucleofected non-integrating episomal vectors in skin fibroblasts of three family members carrying a single nucleotide variant (SNV) in SCN5A, which encodes the cardiac-type sodium channel, and of a related healthy control. The SNV SCN5A_c.4573G > A had been previously identified in a Brugada Syndrome patient. The resulting iPS cell lines differentiate into cells of the 3 germ layers, display normal karyotypes and express pluripotency surface markers and genes. Thus, they are a reliable source to study the effect of the identified mutation in a physiologically relevant environment.