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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Detalles Bibliográficos
Autores: Martínez Moreno, Rebecca, Carreras, David, Aran, Begoña, Kuebler, Bernd, Sarquella Brugada, Georgia, Brugada, Ramon, Pérez, Guillermo J., Scornik, Fabiana S., Selga, Elisabet
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Institución:Universidad de Barcelona
Repositorio:Dipòsit Digital de la UB
OAI Identifier:oai:diposit.ub.edu:2445/188702
Acceso en línea:https://hdl.handle.net/2445/188702
Access Level:acceso abierto
Palabra clave:Cèl·lules mare
Malalties cardiovasculars
Malalties hereditàries
Stem cells
Cardiovascular diseases
Genetic diseases
Descripción
Sumario: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.