Low IGF-I Bioavailability Impairs Growth and Glucose Metabolism in a Mouse Model of Human PAPPA2 p.Ala1033Val Mutation

M Fujimoto, M Andrew, L Liao, D Zhang… - …, 2019 - academic.oup.com
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 …

PAPPA2 as a Therapeutic Modulator of IGF-I Bioavailability: in Vivo and in Vitro Evidence

M Andrew, L Liao, M Fujimoto, J Khoury… - Journal of the …, 2018 - academic.oup.com
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 …

Anthropometric and biochemical correlates of PAPP-A2, free IGF-I, and IGFBP-3 in childhood

M Fujimoto, JC Khoury, PR Khoury… - European journal of …, 2020 - academic.oup.com
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 …

Pappa2 deletion alters IGFBPs but has little effect on glucose disposal or adiposity

JK Christians, AK Bath, N Amiri - Growth Hormone & IGF Research, 2015 - Elsevier
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 …

Mutations in pregnancy‐associated plasma protein A2 cause short stature due to low IGF‐I availability

A Dauber, MT Muñoz‐Calvo, V Barrios… - EMBO molecular …, 2016 - embopress.org
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 …

PAPP-A2 expression by osteoblasts is required for normal postnatal growth in mice

N Amiri, JK Christians - Growth Hormone & IGF Research, 2015 - Elsevier
Objective Pregnancy associated plasma protein-A2 (PAPP-A2) is a protease that cleaves
insulin-like growth factor binding protein-5 (IGFBP-5), the most abundant IGFBP in bone …

Disorders caused by genetic defects associated with GH-dependent genes: PAPPA2 defects

M Fujimoto, M Andrew, A Dauber - Molecular and cellular endocrinology, 2020 - Elsevier
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 …

Treatment with recombinant human insulin-like growth factor-1 improves growth in patients with PAPP-A2 deficiency

MT Muñoz-Calvo, V Barrios, J Pozo… - The Journal of …, 2016 - academic.oup.com
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 …

Inactivation of insulin-like-growth factors diminished the anabolic effects of pregnancy-associated plasma protein-A (PAPP-A) on bone in mice

D Phang, M Rehage, B Bonafede, D Hou… - Growth Hormone & IGF …, 2010 - Elsevier
In vivo studies have provided ubiquitous evidence that pregnancy-associated plasma
protein-A (PAPP-A) functions as a potent anabolic factor. While some evidence supports the …

Pappa2 deletion has sex-and age-specific effects on bone in mice

JK Christians, N Amiri, JD Schipilow, SW Zhang… - Growth Hormone & IGF …, 2019 - Elsevier
Objective In humans, loss-of-function mutations in the gene encoding pregnancy-associated
pregnancy protein-A2 cause short stature and slightly reduced bone density. The goal of this …