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Elevated ratio of acylated to unacylated ghrelin in children and young adults with Prader-Willi syndrome.
Endocrine. 2015 Dec; 50(3):633-42.E

Abstract

Prader-Willi syndrome (PWS) is characterized by a switch from failure to thrive to excessive weight gain and hyperphagia in early childhood. Hyperghrelinemia may be involved in the underlying mechanisms of the switch. The purpose of this study is to evaluate acylated ghrelin (AG) and unacylated ghrelin (UAG) levels in PWS and investigate their associations with hyperphagia. This is a cross-sectional clinical study conducted in three PWS expert centers in the Netherlands and France. Levels of AG and UAG and the AG/UAG ratio were determined in 138 patients with PWS (0.2-29.4 years) and compared with 50 age-matched obese subjects (4.3-16.9 years) and 39 healthy controls (0.8-28.6 years). AEBSF was used to inhibit deacylation of AG. As a group, PWS patients had higher AG but similar UAG levels as healthy controls (AG 129.1 vs 82.4 pg/ml, p = 0.016; UAG 135.3 vs 157.3 pg/ml, resp.), resulting in a significantly higher AG/UAG ratio (1.00 vs 0.61, p = 0.001, resp.). Obese subjects had significantly lower AG and UAG levels than PWS and controls (40.3 and 35.3 pg/ml, resp.), but also a high AG/UAG ratio (1.16). The reason for the higher AG/UAG ratio in PWS and obese was, however, completely different, as PWS had a high AG and obese a very low UAG. PWS patients without weight gain or hyperphagia had a similar AG/UAG ratio as age-matched controls, in contrast to those with weight gain and/or hyperphagia who had an elevated AG/UAG ratio. The switch to excessive weight gain in PWS seems to coincide with an increase in the AG/UAG ratio, even prior to the start of hyperphagia.

Authors+Show Affiliations

Dutch Growth Research Foundation, Westzeedijk 106, 3016 AH, Rotterdam, The Netherlands. R.Kuppens@kindengroei.nl. Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands. R.Kuppens@kindengroei.nl.Unité D'endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale, Centre de Référence du Syndrome de Prader-Willi, Hôpital des enfants, Toulouse, France.Dutch Growth Research Foundation, Westzeedijk 106, 3016 AH, Rotterdam, The Netherlands. Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.Unité D'endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale, Centre de Référence du Syndrome de Prader-Willi, Hôpital des enfants, Toulouse, France. Axe pédiatrique du CIC 9302/INSERM. Hôpital des enfants, Toulouse, France.Unité D'endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale, Centre de Référence du Syndrome de Prader-Willi, Hôpital des enfants, Toulouse, France.Division of Pediatric Endocrinology, Hôpital Femme-Mère-Enfant, University of Lyon, Bron/Lyon, France.Division of Pediatric Endocrinology, Hôpital Femme-Mère-Enfant, University of Lyon, Bron/Lyon, France.Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands.Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands.Alizé Pharma, 69130, Ecully, France.Alizé Pharma, 69130, Ecully, France.Alizé Pharma, 69130, Ecully, France.Unité D'endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale, Centre de Référence du Syndrome de Prader-Willi, Hôpital des enfants, Toulouse, France. INSERM U1043, Centre de Physiopathologie de Toulouse Purpan, Université Paul Sabatier, Toulouse, France.Dutch Growth Research Foundation, Westzeedijk 106, 3016 AH, Rotterdam, The Netherlands. Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

25989955

Citation

Kuppens, R J., et al. "Elevated Ratio of Acylated to Unacylated Ghrelin in Children and Young Adults With Prader-Willi Syndrome." Endocrine, vol. 50, no. 3, 2015, pp. 633-42.
Kuppens RJ, Diène G, Bakker NE, et al. Elevated ratio of acylated to unacylated ghrelin in children and young adults with Prader-Willi syndrome. Endocrine. 2015;50(3):633-42.
Kuppens, R. J., Diène, G., Bakker, N. E., Molinas, C., Faye, S., Nicolino, M., Bernoux, D., Delhanty, P. J., van der Lely, A. J., Allas, S., Julien, M., Delale, T., Tauber, M., & Hokken-Koelega, A. C. (2015). Elevated ratio of acylated to unacylated ghrelin in children and young adults with Prader-Willi syndrome. Endocrine, 50(3), 633-42. https://doi.org/10.1007/s12020-015-0614-x
Kuppens RJ, et al. Elevated Ratio of Acylated to Unacylated Ghrelin in Children and Young Adults With Prader-Willi Syndrome. Endocrine. 2015;50(3):633-42. PubMed PMID: 25989955.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Elevated ratio of acylated to unacylated ghrelin in children and young adults with Prader-Willi syndrome. AU - Kuppens,R J, AU - Diène,G, AU - Bakker,N E, AU - Molinas,C, AU - Faye,S, AU - Nicolino,M, AU - Bernoux,D, AU - Delhanty,P J D, AU - van der Lely,A J, AU - Allas,S, AU - Julien,M, AU - Delale,T, AU - Tauber,M, AU - Hokken-Koelega,A C S, Y1 - 2015/05/20/ PY - 2015/01/16/received PY - 2015/04/21/accepted PY - 2015/5/21/entrez PY - 2015/5/21/pubmed PY - 2016/9/27/medline KW - AG KW - AG/UAG ratio KW - Acylated ghrelin KW - Ghrelin KW - Prader–Willi syndrome KW - UAG KW - Unacylated ghrelin SP - 633 EP - 42 JF - Endocrine JO - Endocrine VL - 50 IS - 3 N2 - Prader-Willi syndrome (PWS) is characterized by a switch from failure to thrive to excessive weight gain and hyperphagia in early childhood. Hyperghrelinemia may be involved in the underlying mechanisms of the switch. The purpose of this study is to evaluate acylated ghrelin (AG) and unacylated ghrelin (UAG) levels in PWS and investigate their associations with hyperphagia. This is a cross-sectional clinical study conducted in three PWS expert centers in the Netherlands and France. Levels of AG and UAG and the AG/UAG ratio were determined in 138 patients with PWS (0.2-29.4 years) and compared with 50 age-matched obese subjects (4.3-16.9 years) and 39 healthy controls (0.8-28.6 years). AEBSF was used to inhibit deacylation of AG. As a group, PWS patients had higher AG but similar UAG levels as healthy controls (AG 129.1 vs 82.4 pg/ml, p = 0.016; UAG 135.3 vs 157.3 pg/ml, resp.), resulting in a significantly higher AG/UAG ratio (1.00 vs 0.61, p = 0.001, resp.). Obese subjects had significantly lower AG and UAG levels than PWS and controls (40.3 and 35.3 pg/ml, resp.), but also a high AG/UAG ratio (1.16). The reason for the higher AG/UAG ratio in PWS and obese was, however, completely different, as PWS had a high AG and obese a very low UAG. PWS patients without weight gain or hyperphagia had a similar AG/UAG ratio as age-matched controls, in contrast to those with weight gain and/or hyperphagia who had an elevated AG/UAG ratio. The switch to excessive weight gain in PWS seems to coincide with an increase in the AG/UAG ratio, even prior to the start of hyperphagia. SN - 1559-0100 UR - https://www.unboundmedicine.com/medline/citation/25989955/Elevated_ratio_of_acylated_to_unacylated_ghrelin_in_children_and_young_adults_with_Prader_Willi_syndrome_ L2 - https://dx.doi.org/10.1007/s12020-015-0614-x DB - PRIME DP - Unbound Medicine ER -