Pregnancy-associated plasma protein (PAPP)-A2 in physiology and disease

Vicente Barrios, Julie A. Chowen, Álvaro Martín-Rivada, Santiago Guerra-Cantera, Jesús Pozo, Shoshana Yakar, Ron G. Rosenfeld, Luis A. Pérez-Jurado, Juan Suárez, Jesús Argente

Research output: Contribution to journalReview articlepeer-review


The growth hormone (GH)/insulin-like growth factor (IGF) axis plays fundamental roles during development, maturation, and aging. Members of this axis, composed of various ligands, receptors, and binding proteins, are regulated in a tissue-and time-specific manner that requires precise control that is not completely understood. Some of the most recent advances in understanding the implications of this axis in human growth are derived from the identifications of new mutations in the gene encoding the pregnancy-associated plasma protein PAPP-A2 protease that liberates IGFs from their carrier proteins in a selective manner to allow binding to the IGF receptor 1. The identification of three nonrelated families with mutations in the PAPP-A2 gene has shed light on how this protease affects human physiology. This review summarizes our understanding of the implications of PAPP-A2 in growth physiology, obtained from studies in genetically modified animal models and the PAPP-A2 deficient patients known to date.

Original languageEnglish (US)
Article number3576
Issue number12
StatePublished - Dec 2021


  • Growth hormone axis
  • IGF1
  • IGF2
  • IGFBPs
  • PAPP-A
  • PAPP-A2
  • STC1
  • STC2

ASJC Scopus subject areas

  • General Biochemistry, Genetics and Molecular Biology


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