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Generation of two iPSC lines (FAMRCi004-A and FAMRCi004-B) from patient with familial progressive cardiac conduction disorder carrying genetic variant DSP p.His1684Arg.
Khudiakov, Aleksandr; Perepelina, Kseniya; Klauzen, Polina; Zlotina, Anna; Gusev, Konstantin; Kaznacheyeva, Elena; Malashicheva, Anna; Kostareva, Anna.
Affiliation
  • Khudiakov A; Almazov National Medical Research Centre, Saint-Petersburg, Russian Federation. Electronic address: khudyakov_aa@almazovcentre.ru.
  • Perepelina K; Almazov National Medical Research Centre, Saint-Petersburg, Russian Federation; Saint Petersburg State University, Saint-Petersburg, Russian Federation.
  • Klauzen P; Almazov National Medical Research Centre, Saint-Petersburg, Russian Federation; Saint Petersburg State University, Saint-Petersburg, Russian Federation; Institute of Cytology RAS, Saint-Petersburg, Russian Federation.
  • Zlotina A; Almazov National Medical Research Centre, Saint-Petersburg, Russian Federation.
  • Gusev K; Institute of Cytology RAS, Saint-Petersburg, Russian Federation.
  • Kaznacheyeva E; Institute of Cytology RAS, Saint-Petersburg, Russian Federation.
  • Malashicheva A; Almazov National Medical Research Centre, Saint-Petersburg, Russian Federation; Saint Petersburg State University, Saint-Petersburg, Russian Federation; Institute of Cytology RAS, Saint-Petersburg, Russian Federation.
  • Kostareva A; Almazov National Medical Research Centre, Saint-Petersburg, Russian Federation.
Stem Cell Res ; 43: 101720, 2020 03.
Article in En | MEDLINE | ID: mdl-32062131
ABSTRACT
Human iPSC cell lines (FAMRCi004-A and FAMRCi004-B) were generated from patient with progressive cardiac conduction disease and sick sinus syndrome carrying DSP p.His1684Arg genetic variant. Patient-specific adipose tissue-derived mesenchymal multipotent stromal cells were reprogrammed using non-integrative Sendai viruses. Established iPSC lines showed normal karyotype, expressed pluripotent markers and were able to differentiate toward three germ layers in vitro. The reported iPSC lines could be useful tool for in vitro modeling of progressive cardiac conduction disease associated with mutations in desmosomal genes.
Subject(s)

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Desmoplakins / Induced Pluripotent Stem Cells / Cardiac Conduction System Disease / Heart Block Limits: Humans Language: En Journal: Stem Cell Res Year: 2020 Document type: Article

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Desmoplakins / Induced Pluripotent Stem Cells / Cardiac Conduction System Disease / Heart Block Limits: Humans Language: En Journal: Stem Cell Res Year: 2020 Document type: Article