Generation of a human induced pluripotent stem cell line NTUHi002-A from a patient with aceruloplasminemia harboring a homozygous splicing mutation c.607+1 delG in CP gene

Stem Cell Res. 2022 Aug:63:102856. doi: 10.1016/j.scr.2022.102856. Epub 2022 Jul 8.

Abstract

Aceruloplasminemia is a rare autosomal recessive disorder caused by mutations in the CP gene, encoding the copper-binding protein ceruloplasmin. A mutation in the CP gene results in brain and systemic iron overload, which is classified as a rare subtype of neurodegeneration with brain iron accumulation (NBIA). Here, we used the Sendai virus delivery system to generate induced pluripotent stem cells from peripheral blood mononuclear cells of a patient carrying the CP c.607+1 delG homozygous splicing mutation. The generated cell line retained the original genotype, expressed pluripotency markers, and differentiated into cells of the three germ layers.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Line*
  • Ceruloplasmin* / deficiency
  • Ceruloplasmin* / genetics
  • Humans
  • Induced Pluripotent Stem Cells*
  • Iron Metabolism Disorders
  • Leukocytes, Mononuclear
  • Mutation
  • Neurodegenerative Diseases

Substances

  • Ceruloplasmin

Supplementary concepts

  • Familial apoceruloplasmin deficiency