{"id":"43be4179-48a0-5691-831e-9a5135c14f4f","stable_key":"6cdb37aa-8998-5ea8-829d-4f995caf98fc:l-carnitine-organic-acid-loss","predicate":"urinary_loss_associated_with","statement":"Four patients with propionic or methylmalonic aciduria had low plasma free carnitine and elevated short-chain acylcarnitine excretion; muscle carnitine was low in two biopsied patients.","claim_class":"observational","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"dcd1831d-4b19-55a3-ac60-34e443cbf600","mechanism_event_label":"A metabolic block can tie up carnitine and lead to its loss in urine.","subject":{"id":"a8093e02-6e69-592d-a171-f0821c802e28","slug":"propionylcarnitine","display_name":"Propionylcarnitine","entity_type_key":"small_molecule"},"object":{"id":"0301f11b-b398-5c00-bf04-f88922bcecf3","slug":"human-free-carnitine-organic-acidemia","display_name":"Human free carnitine pools in organic acidemias","entity_type_key":"cellular_process"},"evidence_count":1,"mechanism_event":{"id":"dcd1831d-4b19-55a3-ac60-34e443cbf600","stable_key":"6cdb37aa-8998-5ea8-829d-4f995caf98fc:l-carnitine-organic-acid-loss-event","event_type":"observed_relationship","label":"A metabolic block can tie up carnitine and lead to its loss in urine.","description":"Four patients with propionic or methylmalonic aciduria had low plasma free carnitine and elevated short-chain acylcarnitine excretion; muscle carnitine was low in two biopsied patients.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"a8093e02-6e69-592d-a171-f0821c802e28","slug":"propionylcarnitine","display_name":"Propionylcarnitine","entity_type_key":"small_molecule"},"role":"subject","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"0301f11b-b398-5c00-bf04-f88922bcecf3","slug":"human-free-carnitine-organic-acidemia","display_name":"Human free carnitine pools in organic acidemias","entity_type_key":"cellular_process"},"role":"target","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""},{"entity":{"id":"69ac0570-94d8-59d8-85ef-7f67cf302d16","slug":"propionyl-coa","display_name":"Propionyl-CoA","entity_type_key":"small_molecule"},"role":"context_participant","stoichiometry":null,"state_label":"","sequence_order":2,"notes":""},{"entity":{"id":"6e34c035-9371-578f-b799-fd2a14e9e40f","slug":"l-carnitine","display_name":"L-Carnitine","entity_type_key":"small_molecule"},"role":"context_participant","stoichiometry":null,"state_label":"","sequence_order":3,"notes":""}]},"contexts":[{"dimension":"availability_state","value_text":"nutrient_deficiency","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null},{"dimension":"evidence_access","value_text":"Primary abstract","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"experimental_model","value_text":"Two PA and two MMA cases; plasma, urine and selected muscle measurements.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"Export-driven tissue depletion is the authors' mechanism; these inherited conditions are not synonymous with dietary B12 deficiency.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"nutrient_topic","value_text":"L-Carnitine collection; isomer, preparation, species, exposure and manipulation remain explicit.","comparator":null,"unit":null,"notes":"","entity":{"slug":"l-carnitine","display_name":"L-Carnitine","entity_type_key":"small_molecule"}},{"dimension":"plain_language","value_text":"A metabolic block can tie up carnitine and lead to its loss in urine.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency. · 1984 · https://pubmed.ncbi.nlm.nih.gov/6723070/ · DOI 10.1016/0009-8981(84)90187-6","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"trigger_kind","value_text":"nutrient_deficiency","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null}],"evidence":[{"id":"756b0df5-a4f2-5f20-8cbc-895455138cc4","evidence_kind":"source_excerpt","locator":"Lines 258-264","start_line":258,"end_line":264,"excerpt":"## l-carnitine-organic-acid-loss\nA metabolic block can tie up carnitine and lead to its loss in urine.\nFour patients with propionic or methylmalonic aciduria had low plasma free carnitine and elevated short-chain acylcarnitine excretion; muscle carnitine was low in two biopsied patients.\nModel: Two PA and two MMA cases; plasma, urine and selected muscle measurements.\nLimitations: Export-driven tissue depletion is the authors' mechanism; these inherited conditions are not synonymous with dietary B12 deficiency.\nEvidence access: Primary abstract\nPropionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency. · 1984 · https://pubmed.ncbi.nlm.nih.gov/6723070/ · DOI 10.1016/0009-8981(84)90187-6","model_system":"Two PA and two MMA cases; plasma, urine and selected muscle measurements.","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":"e2c9c42b-806a-55e2-abde-b0320ea94704","stable_key":"import-6cdb37aa-8998-5ea8-829d-4f995caf98fc","title":"L-Carnitine: synthesis, acyl-group transport, fuel selection and nutrient interactions (2026-09-19)","document_type":"imported_text","citation_label":"AI-assisted research curation; primary-abstract references and experimental limitations individually identified. 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