{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T11:04:09Z","timestamp":1790420649991,"version":"4.1.0"},"reference-count":30,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2020,3,2]],"date-time":"2020-03-02T00:00:00Z","timestamp":1583107200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Girls with Turner syndrome (TS) are at increased risk of developing insulin resistance and coronary artery disease as a result of hypertension and obesity frequently seen in these patients. On the other hand, it is known that obesity is associated with increased serum levels of branched-chain amino acids (BCAAs: valine; leucine and isoleucine) and aromatic amino acids. The aim of the study is to compare the metabolic fingerprint of girls with TS to the metabolic fingerprint of girls with obesity. Metabolic fingerprinting using an untargeted metabolomic approach was examined in plasma from 46 girls with TS (study group) and 22 age-matched girls with obesity (control group). The mean values of BCAAs, methionine, phenylalanine, lysine, tryptophan, histidine, tyrosine, alanine and ornithine were significantly lower in the study group than in the control (p from 0.0025 to &lt;0.000001). Strong significant correlation between BCAAs, phenylalanine, arginine, tyrosine, glutamic acid, citrulline and alanine, and body mass index expressed as standard deviation score BMI-SDS in the patients with obesity (p from 0.049 to 0.0005) was found. In contrast; there was no correlation between these amino acids and BMI-SDS in the girls with TS. It is suggested that obesity in patients with TS is not associated with altered amino acids metabolism.<\/jats:p>","DOI":"10.3390\/jcm9030664","type":"journal-article","created":{"date-parts":[[2020,3,2]],"date-time":"2020-03-02T07:50:53Z","timestamp":1583135453000},"page":"664","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Metabolic Fingerprint of Turner Syndrome"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4290-8714","authenticated-orcid":false,"given":"Jolanta","family":"Bugajska","sequence":"first","affiliation":[{"name":"Department of Clinical Biochemistry, Institute of Pediatrics, Jagiellonian University Medical College, Krakow, Wielicka St. 265, 30-663 Krakow, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Berska","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry, Institute of Pediatrics, Jagiellonian University Medical College, Krakow, Wielicka St. 265, 30-663 Krakow, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ma\u0142gorzata","family":"W\u00f3jcik","sequence":"additional","affiliation":[{"name":"Department of Paediatric and Adolescent Endocrinology, Institute of Paediatrics, Jagiellonian University, Medical College, Krakow, Wielicka St. 265, 30-663 Krakow, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerzy B.","family":"Starzyk","sequence":"additional","affiliation":[{"name":"Department of Paediatric and Adolescent Endocrinology, Institute of Paediatrics, Jagiellonian University, Medical College, Krakow, Wielicka St. 265, 30-663 Krakow, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krystyna","family":"Sztefko","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry, Institute of Pediatrics, Jagiellonian University Medical College, Krakow, Wielicka St. 265, 30-663 Krakow, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,3,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1530\/eje.1.02267","article-title":"Body composition is distinctly altered in Turner syndrome: Relations to glucose metabolism, circulating adipokines, and endothelial adhesion molecules","volume":"155","author":"Gravholt","year":"2006","journal-title":"Eur. J. Endocrinol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1046\/j.1365-2265.1998.00552.x","article-title":"The impact of obesity on cardiovascular risk factors in Turner\u2019s syndrome","volume":"49","author":"Elsheikh","year":"1998","journal-title":"Clin. Endocrinol. (Oxf.)"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/S0895-4356(97)00237-0","article-title":"Morbidity in Turner syndrome","volume":"51","author":"Gravholt","year":"1998","journal-title":"J. Clin. Epidemiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1016\/j.bbacli.2015.04.005","article-title":"Cardiometabolic and vascular risks in young and adolescent girls with Turner syndrome","volume":"3","author":"Mavinkurve","year":"2015","journal-title":"BBA Clin."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1210\/jc.2006-0158","article-title":"Clinical significance of the parental origin of the X chromosome in turner syndrome","volume":"92","author":"Sagi","year":"2007","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"2109","DOI":"10.1210\/jc.2007-2266","article-title":"Reduced abdominal adiposity and improved glucose tolerance in growth hormone-Treated girls with Turner syndrome","volume":"93","author":"Wooten","year":"2008","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"E1025","DOI":"10.1210\/jc.2010-3023","article-title":"Prevalence and incidence of diabetes mellitus in GH-Treated children and adolescents: Analysis from the GeNeSIS observational research program","volume":"96","author":"Child","year":"2011","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.metabol.2015.10.027","article-title":"Abdominal obesity and circulating metabolites: A twin study approach","volume":"65","author":"Bogl","year":"2016","journal-title":"Metabolism"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1186\/s12986-015-0059-5","article-title":"Associations among the plasma amino acid profile, obesity, and glucose metabolism in Japanese adults with normal glucose tolerance","volume":"13","author":"Takashina","year":"2016","journal-title":"Nutr. Metab. (Lond.)"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1038\/nm.2307","article-title":"Metabolite profiles and the risk of developing diabetes","volume":"17","author":"Wang","year":"2011","journal-title":"Nat. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/j.numecd.2016.03.013","article-title":"Branched-Chain and aromatic amino acids, insulin resistance and liver specific ectopic fat storage in overweight to obese subjects","volume":"26","author":"Haufe","year":"2016","journal-title":"Nutr. Metab. Cardiovasc. Dis."},{"key":"ref_12","first-page":"18","article-title":"Somatic development indices in children and youth of Warsaw","volume":"5","author":"Palczewska","year":"2001","journal-title":"Med. Wieku Rozwoj."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"e500","DOI":"10.1542\/peds.2004-1921","article-title":"Homeostasis model assessment is more reliable than the fasting glucose\/insulin ratio and quantitative insulin sensitivity check index for assessing insulin resistance among obese children and adolescents","volume":"115","author":"Keskin","year":"2005","journal-title":"Pediatrics"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1455","DOI":"10.1210\/jc.2002-020542","article-title":"Growth hormone replacement therapy induces insulin resistance by activating the glucose-Fatty acid cycle","volume":"88","author":"Bramnert","year":"2003","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_15","first-page":"769","article-title":"Carbohydrate metabolism during long-Term growth hormone (GH) treatment and after discontinuation of GH treatment in girls with Turner syndrome participating in a randomized dose-Response study. Dutch Advisory Group on Growth Hormone","volume":"85","author":"Sas","year":"2000","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1016\/j.ghir.2008.09.001","article-title":"Amino acid profiles in adults with growth hormone (GH) deficiency before and during GH replacement therapy","volume":"19","author":"Fernholm","year":"2009","journal-title":"Growth Horm. IGF Res."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/0026-0495(91)90116-E","article-title":"Growth hormone improves muscle protein metabolism and whole body nitrogen economy in man during a hyponitrogenous diet","volume":"40","author":"Lundeberg","year":"1991","journal-title":"Metabolism"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1007\/s00726-008-0152-4","article-title":"Amino acids and gut function","volume":"37","author":"Wang","year":"2009","journal-title":"Amino Acids."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1007\/s11306-018-1393-1","article-title":"Serum metabolomic profiles associated with postmenopausal hormone use","volume":"14","author":"Stevens","year":"2018","journal-title":"Metabolomics"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/j.1365-2265.2012.04374.x","article-title":"Effects of oestrogen and testosterone therapy on serum metabolites in postmenopausal women","volume":"77","author":"Zang","year":"2012","journal-title":"Clin. Endocrinol. (Oxf.)"},{"key":"ref_21","unstructured":"Carpenter, K. (2020, January 02). Available online: http:\/\/link.springer.com\/10.1007\/978-1-4939-1923-9."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Yoon, M.-S. (2016). The Emerging Role of Branched-Chain Amino Acids in Insulin Resistance and Metabolism. Nutrient, 8.","DOI":"10.3390\/nu8070405"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/j.numecd.2018.01.001","article-title":"Amino acid profiles of young adults differ by sex, body mass index and insulin resistance","volume":"28","author":"Rosado","year":"2018","journal-title":"Nutr. Metab. Cardiovasc. Dis."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"10635","DOI":"10.1074\/jbc.M115.706200","article-title":"The Methionine Transamination Pathway Controls Hepatic Glucose Metabolism through Regulation of the GCN5 Acetyltransferase and the PGC-1alpha Transcriptional Coactivator","volume":"291","author":"Tavares","year":"2016","journal-title":"J. Biol. Chem."},{"key":"ref_25","doi-asserted-by":"crossref","unstructured":"Ables, G.P., Perrone, C.E., Orentreich, D., and Orentreich, N. (2012). Methionine-Restricted C57BL\/6J mice are resistant to diet-Induced obesity and insulin resistance but have low bone density. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0051357"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"3721","DOI":"10.2337\/db14-0464","article-title":"Mechanisms of increased in vivo insulin sensitivity by dietary methionine restriction in mice","volume":"63","author":"Stone","year":"2014","journal-title":"Diabetes"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1016\/j.cmet.2009.02.002","article-title":"A branched-Chain amino acid-Related metabolic signature that differentiates obese and lean humans and contributes to insulin resistance","volume":"9","author":"Newgard","year":"2009","journal-title":"Cell Metab."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1210\/jcem-72-4-832","article-title":"Insulin resistance: An early metabolic defect of Turner\u2019s syndrome","volume":"72","author":"Caprio","year":"1991","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"R1387","DOI":"10.1152\/ajpregu.00159.2004","article-title":"Glycine intake decreases plasma free fatty acids, adipose cell size, and blood pressure in sucrose-Fed rats","volume":"287","author":"Perez","year":"2004","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1659","DOI":"10.1007\/s00726-014-1715-1","article-title":"Scallop protein with endogenous high taurine and glycine content prevents high-Fat, high-Sucrose-Induced obesity and improves plasma lipid profile in male C57BL\/6J mice","volume":"46","author":"Tastesen","year":"2014","journal-title":"Amino Acids"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/3\/664\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T09:03:15Z","timestamp":1760173395000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/3\/664"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,2]]},"references-count":30,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2020,3]]}},"alternative-id":["jcm9030664"],"URL":"https:\/\/doi.org\/10.3390\/jcm9030664","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,3,2]]}}}