{"id":"bde37a68-88b7-5178-a2ec-45a0fcf6ef38","stable_key":"c3df3634-4c3a-5099-a5d9-e4f6344c1084:echs1-crotonyl-coa-hydration","predicate":"converted_to","statement":"Short-chain enoyl-CoA hydratase ECHS1 hydrates trans-crotonyl-CoA to (S)-3-hydroxybutyryl-CoA.","claim_class":"mechanistic","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"f2a4353b-8676-5a91-968e-1da496af58cb","mechanism_event_label":"Water is added across the four-carbon intermediate's double bond.","subject":{"id":"c8ce07a8-dfe0-5fb9-9529-f4fe2fddc134","slug":"crotonyl-coa","display_name":"Crotonyl-CoA","entity_type_key":"small_molecule"},"object":{"id":"888ec305-791f-5233-b33d-3351058fddcc","slug":"s-3-hydroxybutyryl-coa","display_name":"(S)-3-Hydroxybutyryl-CoA","entity_type_key":"small_molecule"},"evidence_count":1,"mechanism_event":{"id":"f2a4353b-8676-5a91-968e-1da496af58cb","stable_key":"c3df3634-4c3a-5099-a5d9-e4f6344c1084:echs1-crotonyl-coa-hydration-event","event_type":"biochemical_relationship","label":"Water is added across the four-carbon intermediate's double bond.","description":"Short-chain enoyl-CoA hydratase ECHS1 hydrates trans-crotonyl-CoA to (S)-3-hydroxybutyryl-CoA.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"23e1797b-7992-5004-85c8-2a1ada4bf806","slug":"echs1","display_name":"Short-chain enoyl-CoA hydratase / ECHS1","entity_type_key":"protein"},"role":"catalyst","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"c8ce07a8-dfe0-5fb9-9529-f4fe2fddc134","slug":"crotonyl-coa","display_name":"Crotonyl-CoA","entity_type_key":"small_molecule"},"role":"substrate","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""},{"entity":{"id":"d31225b7-8e94-5857-9ea1-67ac794b1f15","slug":"water","display_name":"H2O","entity_type_key":"small_molecule"},"role":"cosubstrate","stoichiometry":null,"state_label":"","sequence_order":2,"notes":""},{"entity":{"id":"888ec305-791f-5233-b33d-3351058fddcc","slug":"s-3-hydroxybutyryl-coa","display_name":"(S)-3-Hydroxybutyryl-CoA","entity_type_key":"small_molecule"},"role":"product","stoichiometry":null,"state_label":"","sequence_order":3,"notes":""}]},"contexts":[{"dimension":"experimental_model","value_text":"Crotonyl-CoA hydratase assays in control human fibroblasts","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"This shared short-chain reaction can process lysine-derived crotonyl-CoA, but the assay does not trace its carbon from lysine. Disease results are not used to label the control reaction as machinery impairment.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"organism","value_text":"Homo sapiens","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"plain_language","value_text":"Water is added across the four-carbon intermediate's double bond.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"[ferdinandusse2015] Clinical and biochemical characterization of four patients with mutations in ECHS1 (2015). https://link.springer.com/article/10.1186/s13023-015-0290-1 DOI: 10.1186/s13023-015-0290-1","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"tissue_or_cell_type","value_text":"Fibroblasts; mitochondrial enzyme","comparator":null,"unit":null,"notes":"","entity":null}],"evidence":[{"id":"deb59894-ea3b-562d-b580-c88c8c91e471","evidence_kind":"source_excerpt","locator":"Lines 357-365","start_line":357,"end_line":365,"excerpt":"### echs1-crotonyl-coa-hydration\nShort-chain enoyl-CoA hydratase ECHS1 hydrates trans-crotonyl-CoA to (S)-3-hydroxybutyryl-CoA.\nPlain language: Water is added across the four-carbon intermediate's double bond.\nCondition category: normal\norganism: Homo sapiens\ntissue_or_cell_type: Fibroblasts; mitochondrial enzyme\nexperimental_model: Crotonyl-CoA hydratase assays in control human fibroblasts\nlimitations: This shared short-chain reaction can process lysine-derived crotonyl-CoA, but the assay does not trace its carbon from lysine. Disease results are not used to label the control reaction as machinery impairment.\n[ferdinandusse2015] Clinical and biochemical characterization of four patients with mutations in ECHS1 (2015). https://link.springer.com/article/10.1186/s13023-015-0290-1 DOI: 10.1186/s13023-015-0290-1","model_system":"Crotonyl-CoA hydratase assays in control human fibroblasts","directness":"author_interpretation","verification_status":"source_derived_draft","notes":"Exact quote from the accompanying curation document, not from publisher text. Original study references: [ferdinandusse2015] Clinical and biochemical characterization of four patients with mutations in ECHS1 (2015). https://link.springer.com/article/10.1186/s13023-015-0290-1 DOI: 10.1186/s13023-015-0290-1","relationship":"supports","weight":1.0,"link_notes":"","source":{"id":"7633bde7-dcc9-5086-91c6-a45eb96857f3","stable_key":"import-c3df3634-4c3a-5099-a5d9-e4f6344c1084","title":"L-Lysine: mechanism-first literature curation (2026-09-17)","document_type":"imported_text","citation_label":"AI-assisted literature curation; primary study URLs and scope retained in the document and extraction. Not publisher full text.","file_path":"","sha256":"93998d47c21525409ba82f1c82ededf2893dff15fbe61c7deffc175b0e298e97","revision_id":"3897e31f-6624-59e1-a053-8a81b7361632","review_status":"unverified_draft","notes":""}}],"relations":[],"conflicts":[],"corrections":[],"research":null}