Generation of induced pluripotent stem cells MMCi001-A from a Taiwanese hearing loss patient carrying GJB2 pV37I mutation

Stem Cell Res. 2020 Jan:42:101692. doi: 10.1016/j.scr.2019.101692. Epub 2019 Dec 18.

Abstract

Hearing loss is the most common disorder in the sensory system. Mutations in GJB2 have been reported to be very common in sensorineural hearing loss patients. In this report, we generated an induced pluripotent stem cell (iPSC) line, MMCi001-A, from the peripheral blood mononuclear cells of a 4-year-old male hearing loss patient carrying GJB2 pV37I mutation by using the Sendai virus delivery system. The generated iPSCs were demonstrated to express pluripotent markers and be differentiated into three germ layers in vitro and in vivo. This GJB2-pV37I iPSCs is valuable for studying the pathogenic mechanisms and drug discovery of hearing loss.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line
  • Child, Preschool
  • Connexin 26
  • Connexins / metabolism*
  • Hearing Loss / genetics*
  • Humans
  • Induced Pluripotent Stem Cells / metabolism*
  • Male
  • Taiwan

Substances

  • Connexins
  • GJB2 protein, human
  • Connexin 26