Related Experiment Video

Updated: Jul 7, 2025

A High-content In Vitro Pancreatic Islet β-cell Replication Discovery Platform
09:35

A High-content In Vitro Pancreatic Islet β-cell Replication Discovery Platform

Published on: July 16, 2016

11.2K

Increased IGFBP Proteolysis, IGF-I Bioavailability, and Pappalysin Levels in Children With Prader-Willi Syndrome

Vicente Barrios1,2, Álvaro Martín-Rivada1, Gabriel Á Martos-Moreno1,2,3

  • 1Departments of Pediatrics & Pediatric Endocrinology, Hospital Infantil Universitario Niño Jesús, Research Institute "La Princesa", Madrid 28009, Spain.

The Journal of Clinical Endocrinology and Metabolism
|December 23, 2023
PubMed

Insights

Children with Prader-Willi syndrome (PWS) show altered insulin-like growth factor (IGF) axis components. Increased pappalysin (PAPP-A) may enhance IGF-I bioavailability, impacting growth in PWS patients.

Area of Science:

  • Endocrinology
  • Pediatrics
  • Genetics

Background:

  • Prader-Willi syndrome (PWS) is linked to impaired growth hormone (GH) secretion and reduced insulin-like growth factor (IGF)-I levels.
  • Pappalysins (PAPP-A, PAPP-A2) and stanniocalcins (STC-1, STC-2) modulate IGF binding protein (IGFBP) cleavage and IGF bioavailability, but their role in PWS is unclear.

Purpose of the Study:

  • To determine serum levels of PAPP-As and STCs in relation to IGF axis components in prepubertal and pubertal PWS patients.
  • To analyze the effects of GH treatment on these markers in children with PWS.

Main Methods:

  • Studied 40 children and adolescents with PWS and 120 matched controls.
  • Assessed serum levels of IGF-I, IGFBPs, PAPP-As, STCs, and acid-labile subunit.
  • Evaluated the impact of 6-month GH treatment in 11 PWS children.

Main Results:

  • PWS patients exhibited lower total IGF-I, IGFBP-3, and STC-1, but higher free IGF-I, IGFBP-5, and PAPP-A.
  • Pubertal PWS patients showed decreased total IGF-I, IGFBP-3, and intact IGFBP-4, with increased total IGFBP-4 and STCs.
  • GH treatment normalized total IGF-I, IGFBP-3, and IGFBP-4, without affecting PAPP-As or STCs.

Conclusions:

  • Elevated PAPP-A in PWS may enhance IGF-I bioavailability through increased IGFBP proteolysis.
  • Further research is needed to clarify the interplay between growth, GH resistance, and IGF axis dynamics in PWS.
  • Findings suggest PAPP-A's potential role in the pathophysiology of growth disturbances in Prader-Willi syndrome.
Abstract

Keywords:
GH treatmentIGFBPPAPP-APrader-Willi syndromeSTC-1free IGF-I

More Related Videos

A RAPID Method for Blood Processing to Increase the Yield of Plasma Peptide Levels in Human Blood11:36

A RAPID Method for Blood Processing to Increase the Yield of Plasma Peptide Levels in Human Blood

Published on: April 28, 2016

9.4K
Mouse In Vivo Placental Targeted CRISPR Manipulation07:39

Mouse In Vivo Placental Targeted CRISPR Manipulation

Published on: April 14, 2023

2.6K

Related Concept Videos

Insulin Secretory Vesicles01:05

Insulin Secretory Vesicles

Insulin secretory vesicles release insulin to stimulate blood glucose uptake and regulate carbohydrate metabolism. When the blood glucose levels increase, glucose enters the pancreatic β-islet cells through glucose transporters. Once inside, glucose is metabolized through glycolysis, the citric acid cycle, and the electron transport chain, producing ATP. This increase in ATP concentration closes ATP-sensitive potassium channels, leading to depolarization of the membrane and the opening of...
5.0K
Glucagon-like Receptor Agonists01:24

Glucagon-like Receptor Agonists

Incretins include glucagon-like peptide-1 (GLP-1) and glucose-dependent insulinotropic polypeptide (GIP), which stimulate insulin secretion post-meals. In type 2 diabetes, GIP's efficacy is reduced, making GLP-1 a viable drug target. GIP originates from preproGIP.
GLP-1, when administered in high doses intravenously, triggers insulin secretion, inhibits glucagon release, slows gastric emptying, reduces food intake, and restores normal insulin secretion. However, its rapid inactivation by...
327
Cell Specific Gene Expression01:58

Cell Specific Gene Expression

Multicellular organisms contain a variety of structurally and functionally distinct cell types, but the DNA in all the cells originated from the same parent cells. The differences in the cells can be attributed to the differential gene expression. Liver cells, whose functions include detoxification of blood, production of bile to metabolize fats, and synthesis of proteins essential for metabolism, must express a specific set of genes to perform their functions. Gene expression also varies with...
13.6K
TGF - β Signaling Pathway01:16

TGF - β Signaling Pathway

The TGF-β signaling pathway regulates cell growth, differentiation, adhesion, motility, and development. TGF-β ligands that induce TGF-β signaling are synthesized in their latent form. Several proteases or cell surface receptors such as integrins act upon the latent form, releasing the active ligand. There are three types of mammalian TGF-βs: (TGF-β1, TGF-β2, and TGF-β3) that bind as homodimers or heterodimers to TGF-β receptors. The TGF-β receptors...
7.4K
Dipeptidyl Peptidase 4 Inhibitors01:23

Dipeptidyl Peptidase 4 Inhibitors

Dipeptidyl peptidase 4 (DPP-4) is a serine protease widely distributed in the body. It's involved in the inactivation of GLP-1 and GIP hormones, which are crucial for insulin regulation. DPP-4 inhibitors, such as sitagliptin (Januvia), saxagliptin (Onglyza), linagliptin (Tradjenta), alogliptin (Nesina), and vildagliptin (Galvus), help increase the proportion of active GLP-1, enhancing insulin secretion. These inhibitors work by competitively binding to DPP-4. This binding causes a...
189
Genomic Imprinting and Inheritance02:30

Genomic Imprinting and Inheritance

Diploid organisms inherit genetic material through chromosomes from both parents. Copies of the same gene are known as alleles. In most cases, both alleles are simultaneously expressed and allow various cellular processes to function optimally. If one of the alleles is missing or mutated, the expression of the other allele can compensate; however, this is not true for all genes.
The expression of some genes depends on which parent passed the gene to the offspring, through a phenomenon known as...
34.5K