Context Pregnancy-associated plasma protein A2 (PAPPA2) is a protease that cleaves IGF-
binding protein (IGFBP)-3 and IGFBP-5, liberating free IGF-I. Five patients from two families …

Bioactive free IGF-I is critically important for growth. The bioavailability of IGF-I is modulated
by the IGF-binding proteins (IGFBPs) and their proteases, such as pregnancy-associated …

Growth hormone (GH) and its mediator, insulin-like growth factor-1 (IGF-1), have long been
recognized as central to human growth physiology. IGF-1 is known to complex with IGF …

Objective Pregnancy-associated plasma protein-A2 (PAPP-A2) is a metalloproteinase that
cleaves IGFBP-3 and IGFBP-5. Human mutations in PAPPA2 result in short stature with a …

Mutations in multiple genes of the growth hormone/IGF‐I axis have been identified in
syndromes marked by growth failure. However, no pathogenic human mutations have been …

Objective Insulin-like growth factor binding proteins (IGFBPs) are involved in glucose and
lipid metabolism, and their actions are modulated by proteases. The aim of this study was to …

Growth hormone (GH) and its mediator, insulin-like growth factor-1 (IGF-1), play a critical
role in human growth. In circulation, IGF-1 is found in a ternary complex with IGF binding …

In 2016 a new syndrome with postnatal short stature and low IGF1 bioavailability caused by
biallelic loss‐of‐function mutations in the gene encoding the metalloproteinase pregnancy …

Context: Pregnancy-associated plasma protein-A2 (PAPP-A2) is a metalloproteinase that
specifically cleaves IGFBP-3 and IGFBP-5. Mutations in the PAPP-A2 gene have recently …

Context: During pregnancy, circulating IGF binding protein-5 (IGFBP-5) undergoes
substantial molecular redistribution from ternary complexes to either binary complexes or the …