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Technological advances have increased therapeutic options, making it challenging to remain up to date. A centralized therapy knowledgebase is needed for early diagnosis and targeted treatment. This study aimed to identify all treatable IEMs through a scoping literature review, followed by data extraction and analysis according to the Treatabolome principles. Knowledge of treatable IEMs, therapeutic categories, efficacy, and evidence was integrated into the Inborn Errors of Metabolism Knowledgebase (IEMbase), an online database encompassing all IEMs. The study identified 275 treatable IEMs, 18% of all currently known 1564 IEMs, according to the International Classification of Inherited Metabolic Disorders. <jats:italic>Disorders of fatty acid and ketone body metabolism<\/jats:italic> had the highest treatability (67%), followed by <jats:italic>disorders of vitamin and cofactor metabolism<\/jats:italic> (60%), and <jats:italic>disorders of lipoprotein metabolism<\/jats:italic> (42%). The most common treatment strategies were pharmacological therapy (34%), nutritional therapy (34%), and vitamin and trace element supplementation (12%). Treatment effects were most commonly observed in nervous system abnormalities (34%), metabolism\/homeostasis abnormalities (33%), and growth (7%). Predominant evidence sources included case reports with evidence levels 4 (48%) and 5 (12%), and individual cohort studies with evidence level 2b (12%). Our study generated the Metabolic Treatabolome 2024. IEMs are the largest group of monogenic disorders amenable to disease\u2010modifying therapy. With drug repurposing efforts and advancements in gene therapies, this number will expand. IEMbase now provides up\u2010to\u2010date, comprehensive information on clinical and biochemical symptoms and therapeutic options, empowering patients, families, healthcare professionals, and researchers in improving patient outcomes.<\/jats:p>","DOI":"10.1002\/jimd.12835","type":"journal-article","created":{"date-parts":[[2025,1,8]],"date-time":"2025-01-08T01:54:49Z","timestamp":1736301289000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["The Metabolic Treatabolome and <scp>Inborn Errors of Metabolism Knowledgebase<\/scp> therapy tool: Do not miss the opportunity to treat!"],"prefix":"10.1002","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1544-2402","authenticated-orcid":false,"given":"Bibiche","family":"den Hollander","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Emma Children's Hospital Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam UMC Location University of Amsterdam  Amsterdam The Netherlands"},{"name":"Emma Center for Personalized Medicine Amsterdam UMC  Amsterdam The Netherlands"},{"name":"United for Metabolic Diseases  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3086-8447","authenticated-orcid":false,"given":"Eva M. 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