A PAX5-OCT4-PRDM1 developmental switch specifies human primordial germ cells

Nat Cell Biol. 2018 Jun;20(6):655-665. doi: 10.1038/s41556-018-0094-3. Epub 2018 Apr 30.

Abstract

Dysregulation of genetic pathways during human germ cell development leads to infertility. Here, we analysed bona fide human primordial germ cells (hPGCs) to probe the developmental genetics of human germ cell specification and differentiation. We examined the distribution of OCT4 occupancy in hPGCs relative to human embryonic stem cells (hESCs). We demonstrated that development, from pluripotent stem cells to germ cells, is driven by switching partners with OCT4 from SOX2 to PAX5 and PRDM1. Gain- and loss-of-function studies revealed that PAX5 encodes a critical regulator of hPGC development. Moreover, an epistasis analysis indicated that PAX5 acts upstream of OCT4 and PRDM1. The PAX5-OCT4-PRDM1 proteins form a core transcriptional network that activates germline and represses somatic programmes during human germ cell differentiation. These findings illustrate the power of combined genome editing, cell differentiation and engraftment for probing human developmental genetics that have historically been difficult to study.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Cell Differentiation* / genetics
  • Cell Line
  • Gene Editing / methods
  • Gene Expression Regulation, Developmental
  • Human Embryonic Stem Cells / metabolism*
  • Human Embryonic Stem Cells / transplantation
  • Humans
  • Male
  • Mice, Nude
  • Octamer Transcription Factor-3 / genetics
  • Octamer Transcription Factor-3 / metabolism*
  • PAX5 Transcription Factor / genetics
  • PAX5 Transcription Factor / metabolism*
  • Positive Regulatory Domain I-Binding Factor 1 / genetics
  • Positive Regulatory Domain I-Binding Factor 1 / metabolism*
  • Protein Binding
  • SOXB1 Transcription Factors / genetics
  • SOXB1 Transcription Factors / metabolism
  • Signal Transduction
  • Spermatozoa / metabolism*
  • Testis / embryology
  • Testis / metabolism*
  • Time Factors
  • Transcription, Genetic

Substances

  • Octamer Transcription Factor-3
  • PAX5 Transcription Factor
  • PAX5 protein, human
  • POU5F1 protein, human
  • SOX2 protein, human
  • SOXB1 Transcription Factors
  • PRDM1 protein, human
  • Positive Regulatory Domain I-Binding Factor 1