Establishment of heterozygous and homozygous SHANK3 knockout clonal pluripotent stem cells from the parental hESC line SA001 using CRISPR/Cas9

Stem Cell Res. 2023 Oct:72:103209. doi: 10.1016/j.scr.2023.103209. Epub 2023 Sep 22.

Abstract

Phelan-McDermid syndrome (PMS) is a rare genetic disease characterized by a global developmental delay with autism spectrum disorder. PMS is caused by loss of function mutations in the SHANK3 gene leading to SHANK3 protein haploinsufficiency. This study describes the generation of isogenic clones produced from one male human embryonic stem cell line with deletions in SHANK3, in a heterozygous or homozygous manner, using CRISPR/Cas9 indel methodology. Differentiation of these clones into different neuronal lineages will help understanding PMS etiology and find treatments for PMD patients. (85/100 words).

MeSH terms

  • Autism Spectrum Disorder* / genetics
  • CRISPR-Cas Systems / genetics
  • Chromosome Deletion
  • Chromosome Disorders
  • Chromosomes, Human, Pair 22
  • Clone Cells / metabolism
  • Human Embryonic Stem Cells* / metabolism
  • Humans
  • Male
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism

Substances

  • Nerve Tissue Proteins
  • SHANK3 protein, human

Supplementary concepts

  • Telomeric 22q13 Monosomy Syndrome