{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T14:03:54Z","timestamp":1771941834214,"version":"3.50.1"},"reference-count":23,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2026,3]]},"DOI":"10.1016\/j.ymgme.2026.109730","type":"journal-article","created":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T00:23:48Z","timestamp":1767831828000},"page":"109730","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"title":["Metformin therapy to facilitate weight loss in adults with classic maple syrup urine disease"],"prefix":"10.1016","volume":"147","author":[{"given":"Ashlin","family":"Rodrigues","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erin","family":"Sweigert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donna","family":"Robinson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makenna","family":"Kregel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joelle","family":"Williamson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alanna","family":"Koehler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sean","family":"Schreckengast","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cara","family":"Forry","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"KaLynn","family":"Loeven","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Puffenberger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexis","family":"McVey","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Hendrickson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Patel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karlla W.","family":"Brigatti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin","family":"Strauss","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grace Loudon","family":"Meier","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"issue":"5","key":"10.1016\/j.ymgme.2026.109730_bb0005","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1542\/peds.14.5.462","article-title":"A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance","volume":"14","author":"Menkes","year":"1954","journal-title":"Pediatrics"},{"issue":"18","key":"10.1016\/j.ymgme.2026.109730_bb0015","doi-asserted-by":"crossref","DOI":"10.3390\/nu16183145","article-title":"The impact of diet on body composition in a cohort of Pediatric and adult patients with maple syrup urine disease","volume":"16","author":"Pretese","year":"2024","journal-title":"Nutrients"},{"issue":"2","key":"10.1016\/j.ymgme.2026.109730_bb0020","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1210\/er.2017-00253","article-title":"The science of obesity management: an Endocrine Society scientific statement","volume":"39","author":"Bray","year":"2018","journal-title":"Endocr. Rev."},{"issue":"3","key":"10.1016\/j.ymgme.2026.109730_bb0025","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.ymgme.2018.09.006","article-title":"Prevalence of comorbid conditions among adult patients diagnosed with phenylketonuria","volume":"125","author":"Burton","year":"2018","journal-title":"Mol. Genet. Metab."},{"issue":"6","key":"10.1016\/j.ymgme.2026.109730_bb0030","doi-asserted-by":"crossref","DOI":"10.3390\/nu14061311","article-title":"Cardiometabolic and nutritional morbidities of a large, adult, PKU cohort from Andalusia","volume":"14","author":"Dios-Fuentes","year":"2022","journal-title":"Nutrients"},{"issue":"4","key":"10.1016\/j.ymgme.2026.109730_bb0035","doi-asserted-by":"crossref","first-page":"892","DOI":"10.3390\/pediatric16040076","article-title":"Growth parameters and prevalence of obesity in PKU patients and peers: is this the right comparison?","volume":"16","author":"Paterno","year":"2024","journal-title":"Pediatr. Rep."},{"issue":"Suppl. 1","key":"10.1016\/j.ymgme.2026.109730_bb0040","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/jhn.12054","article-title":"Body mass index in adult patients with diet-treated phenylketonuria","volume":"26","author":"Robertson","year":"2013","journal-title":"J. Hum. Nutr. Diet."},{"issue":"10","key":"10.1016\/j.ymgme.2026.109730_bb0045","doi-asserted-by":"crossref","DOI":"10.3390\/nu13103443","article-title":"Is the Phenylalanine-Restricted Diet a Risk Factor for Overweight or Obesity in Patients with Phenylketonuria (PKU)? A Systematic Review and Meta-Analysis","volume":"13","author":"Rodrigues","year":"2021","journal-title":"Nutrients"},{"issue":"1","key":"10.1016\/j.ymgme.2026.109730_bb0050","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1186\/s13023-023-02636-2","article-title":"Overweight and obesity in adult patients with phenylketonuria: a systematic review","volume":"18","author":"Tankeu","year":"2023","journal-title":"Orphanet J. Rare Dis."},{"issue":"1","key":"10.1016\/j.ymgme.2026.109730_bb0055","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1186\/s13023-024-03203-z","article-title":"Systematic literature review of the somatic comorbidities experienced by adults with phenylketonuria","volume":"19","author":"Whitehall","year":"2024","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.ymgme.2026.109730_bb0060","series-title":"GeneReviews((R))","article-title":"Maple Syrup Urine Disease","author":"Strauss","year":"1993"},{"issue":"2","key":"10.1016\/j.ymgme.2026.109730_bb0065","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1007\/s13679-019-00335-3","article-title":"Metformin: mechanisms in human obesity and weight loss","volume":"8","author":"Yerevanian","year":"2019","journal-title":"Curr. Obes. Rep."},{"key":"10.1016\/j.ymgme.2026.109730_bb0070","article-title":"Association of Elevated Serum Branched-chain Amino Acid Levels with Longitudinal Skeletal Muscle Loss","volume":"8(2):bvad178","author":"Imai","year":"2024","journal-title":"J. Endocr. Soc."},{"key":"10.1016\/j.ymgme.2026.109730_bb0075","doi-asserted-by":"crossref","first-page":"28775","DOI":"10.1038\/srep28775","article-title":"Metformin inhibits branched-chain amino acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD","volume":"6","author":"Sonnet","year":"2016","journal-title":"Sci. Rep."},{"issue":"4","key":"10.1016\/j.ymgme.2026.109730_bb0080","doi-asserted-by":"crossref","first-page":"731","DOI":"10.2337\/dc11-1299","article-title":"Diabetes prevention program research G: Long-term safety, tolerability, and weight loss associated with metformin in the diabetes prevention program outcomes study","volume":"35","year":"2012","journal-title":"Diabetes Care"},{"issue":"9","key":"10.1016\/j.ymgme.2026.109730_bb0085","doi-asserted-by":"crossref","first-page":"1566","DOI":"10.1007\/s00125-017-4318-z","article-title":"Metformin: historical overview","volume":"60","author":"Bailey","year":"2017","journal-title":"Diabetologia"},{"issue":"3","key":"10.1016\/j.ymgme.2026.109730_bb0090","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/j.ymgme.2020.01.006","article-title":"Branched-chain alpha-ketoacid dehydrogenase deficiency (maple syrup urine disease): treatment, biomarkers, and outcomes","volume":"129","author":"Strauss","year":"2020","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2026.109730_bb0095","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1146\/annurev-genom-082908-150040","article-title":"Genetics, medicine, and the plain people","volume":"10","author":"Strauss","year":"2009","journal-title":"Annu. Rev. Genomics Hum. Genet."},{"issue":"6","key":"10.1016\/j.ymgme.2026.109730_bb0100","doi-asserted-by":"crossref","first-page":"999","DOI":"10.1542\/peds.109.6.999","article-title":"Diagnosis and treatment of maple syrup disease: a study of 36 patients","volume":"109","author":"Morton","year":"2002","journal-title":"Pediatrics"},{"issue":"1","key":"10.1016\/j.ymgme.2026.109730_bb0105","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1086\/316950","article-title":"Gene preference in maple syrup urine disease","volume":"68","author":"Nellis","year":"2001","journal-title":"Am. J. Hum. Genet."},{"issue":"1\u20132","key":"10.1016\/j.ymgme.2026.109730_bb0110","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/S1096-7192(03)00144-6","article-title":"Relationship of causative genetic mutations in maple syrup urine disease with their clinical expression","volume":"80","author":"Nellis","year":"2003","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgme.2026.109730_bib116","series-title":"Glucophage (metformin hydrochloride) tablets and Glucophage XR (metformin hydrochloride) extended-release tabelts: prescribing information","year":"2017"},{"key":"10.1016\/j.ymgme.2026.109730_bb0115","doi-asserted-by":"crossref","first-page":"d517","DOI":"10.2741\/A299","article-title":"Human mutations affecting branched chain alpha-ketoacid dehydrogenase","volume":"3","author":"Danner","year":"1998","journal-title":"Front. Biosci."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719226000132?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719226000132?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T13:12:01Z","timestamp":1771938721000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719226000132"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":23,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["S1096719226000132"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2026.109730","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2026,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Metformin therapy to facilitate weight loss in adults with classic maple syrup urine disease","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgme.2026.109730","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"109730"}}