Generation of a human induced pluripotent stem cell line (FSMi001-A) from fibroblasts of a patient carrying heterozygous mutation in the REEP1 gene

Stem Cell Res. 2024 Sep:79:103472. doi: 10.1016/j.scr.2024.103472. Epub 2024 Jun 11.

Abstract

Hereditary spastic paraplegias (HSPs) a group of rare, clinically, and genetically heterogeneous disorders characterized by progressive degeneration of the corticospinal tract. Among these HSPs, SPG31 is due to autosomal dominant mutations in the receptor expression-enhancing protein 1 (REEP1) gene. Over 80 genes have been associated with HSPs, and the list is constantly growing as research progresses. This study is aimed to create a patient-derived human induced pluripotent stem cell (hiPSC) line with a specific nonsense mutation to better characterize the etiopathogenesis of the disease.

MeSH terms

  • Cell Differentiation
  • Cell Line
  • Fibroblasts* / metabolism
  • Heterozygote*
  • Humans
  • Induced Pluripotent Stem Cells* / metabolism
  • Male
  • Mutation