{"id":"f6b0b406-8e2c-5b1c-8a72-8dd1545d307c","stable_key":"09b23d0d-35e2-51ff-b1aa-b4f9227e4fa2:isoleucine-echs1-boundary","predicate":"is_not_sole_requirement_for","statement":"ECHS1-deficient patients showed a dominant valine-metabolism defect rather than the expected isolated isoleucine block; liver retained tiglyl-CoA hydration capacity.","claim_class":"mechanistic","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"7bad488e-dec7-50bf-b414-b97ea6a4b26f","mechanism_event_label":"A pathway diagram alone can overstate an enzyme as an unavoidable gate.","subject":{"id":"23e1797b-7992-5004-85c8-2a1ada4bf806","slug":"echs1","display_name":"Short-chain enoyl-CoA hydratase / ECHS1","entity_type_key":"protein"},"object":{"id":"f9cf4f47-678e-5322-984e-ff96421d8c35","slug":"human-isoleucine-tiglyl-processing","display_name":"Tiglyl-CoA processing capacity in human tissues","entity_type_key":"cellular_process"},"evidence_count":1,"mechanism_event":{"id":"7bad488e-dec7-50bf-b414-b97ea6a4b26f","stable_key":"09b23d0d-35e2-51ff-b1aa-b4f9227e4fa2:isoleucine-echs1-boundary-event","event_type":"observed_relationship","label":"A pathway diagram alone can overstate an enzyme as an unavoidable gate.","description":"ECHS1-deficient patients showed a dominant valine-metabolism defect rather than the expected isolated isoleucine block; liver retained tiglyl-CoA hydration capacity.","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":"subject","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"f9cf4f47-678e-5322-984e-ff96421d8c35","slug":"human-isoleucine-tiglyl-processing","display_name":"Tiglyl-CoA processing capacity in human tissues","entity_type_key":"cellular_process"},"role":"target","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""},{"entity":{"id":"c85b327e-f132-5d9a-8e4b-c2976fcc0373","slug":"isoleucine","display_name":"L-Isoleucine","entity_type_key":"small_molecule"},"role":"context_participant","stoichiometry":null,"state_label":"","sequence_order":2,"notes":""},{"entity":{"id":"c63a2ebb-71ce-5926-b959-ec47493a0166","slug":"tiglyl-coa","display_name":"Tiglyl-CoA","entity_type_key":"small_molecule"},"role":"context_participant","stoichiometry":null,"state_label":"","sequence_order":3,"notes":""},{"entity":{"id":"ae40617e-3df7-5d48-8dd2-f16a0d3a8a25","slug":"valine","display_name":"L-Valine","entity_type_key":"small_molecule"},"role":"context_participant","stoichiometry":null,"state_label":"","sequence_order":4,"notes":""}]},"contexts":[{"dimension":"availability_state","value_text":"machinery_impairment","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null},{"dimension":"evidence_access","value_text":"Primary full text","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"experimental_model","value_text":"Four-patient biochemical study with tissue enzyme comparisons.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"Does not establish the same backup capacity in every cell type.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"nutrient_topic","value_text":"L-Isoleucine collection; species, compartment, exposure, co-substrates and manipulation remain explicit.","comparator":null,"unit":null,"notes":"","entity":{"slug":"isoleucine","display_name":"L-Isoleucine","entity_type_key":"small_molecule"}},{"dimension":"plain_language","value_text":"A pathway diagram alone can overstate an enzyme as an unavoidable gate.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"Clinical and biochemical characterization of four patients with mutations in ECHS1. · 2015 · https://pubmed.ncbi.nlm.nih.gov/26081110/ · DOI 10.1186/s13023-015-0290-1","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"trigger_kind","value_text":"machinery_impairment","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null}],"evidence":[{"id":"04c12cb1-d513-546f-9764-88c5b0f6ecf5","evidence_kind":"source_excerpt","locator":"Lines 202-208","start_line":202,"end_line":208,"excerpt":"## isoleucine-echs1-boundary\nA pathway diagram alone can overstate an enzyme as an unavoidable gate.\nECHS1-deficient patients showed a dominant valine-metabolism defect rather than the expected isolated isoleucine block; liver retained tiglyl-CoA hydration capacity.\nModel: Four-patient biochemical study with tissue enzyme comparisons.\nLimitations: Does not establish the same backup capacity in every cell type.\nEvidence access: Primary full text\nClinical and biochemical characterization of four patients with mutations in ECHS1. · 2015 · https://pubmed.ncbi.nlm.nih.gov/26081110/ · DOI 10.1186/s13023-015-0290-1","model_system":"Four-patient biochemical study with tissue enzyme comparisons.","directness":"reported_statement","verification_status":"source_derived_draft","notes":"Original curation paraphrase; evidence access and experimental limitations specified.","relationship":"supports","weight":1.0,"link_notes":"","source":{"id":"4b00f118-00fd-5cef-be97-13125eb958e9","stable_key":"import-09b23d0d-35e2-51ff-b1aa-b4f9227e4fa2","title":"L-Isoleucine: transport, translation, catabolism and cross-nutrient mechanisms (2026-09-19)","document_type":"imported_text","citation_label":"AI-assisted research curation; primary references, access levels and experimental limitations individually identified. Not publisher full text.","file_path":"","sha256":"2058ff0da379586994e7fda6ae0086c60fa3173542bfaf1fb3871ad3e09f9c6c","revision_id":"f32192d4-9913-5d35-acbc-cf90ff17995e","review_status":"unverified_draft","notes":""}}],"relations":[],"conflicts":[],"corrections":[],"research":null}