Generation of two iPS cell lines (FRIMOi003-A and FRIMOi004-A) derived from Stargardt patients carrying ABCA4 compound heterozygous mutations

Stem Cell Res. 2019 Apr:36:101389. doi: 10.1016/j.scr.2019.101389. Epub 2019 Feb 13.

Abstract

Recessive Stargardt disease (STGD1) is an autosomal recessive retinal dystrophy, caused by mutations in the retina-specific ATP-binding cassette transporter (ABCA4) gene, which plays a role as a retinaldehyde flippase in the photoreceptor outer segments. In this work, two human induced pluripotent stem cell (iPSC) lines were generated from STGD1 patients carrying compound heterozygous mutations in ABCA4. Skin fibroblasts were reprogrammed with the Yamanaka factors using a non-integrating, Sendai virus-based approach. Both iPSC lines displayed typical embryonic stem cell morphology, had normal karyotype, expressed several pluripotency markers and were able to differentiate into all three germ layers. Resource table.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • ATP-Binding Cassette Transporters / genetics*
  • Cell Line*
  • DNA Mutational Analysis
  • Fibroblasts
  • Heterozygote
  • Humans
  • Induced Pluripotent Stem Cells*
  • Karyotype
  • Mutation
  • Stargardt Disease / genetics*

Substances

  • ABCA4 protein, human
  • ATP-Binding Cassette Transporters