{"id":"2469f388-593d-5d86-bda5-965eb39f7394","stable_key":"dc8975b1-95ff-5d9b-be17-a1c04610cca7:mo-cpmp-type-a","predicate":"restored_biomarkers_in_type_a","statement":"Urinary S-sulfocysteine, xanthine and urate approached normal within two days in all 11 MoCD-A patients receiving cPMP; eight rapidly improved clinically.","claim_class":"observational","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"90265e41-9483-5a09-9ccd-d78554747545","mechanism_event_label":"Supplying the missing precursor can bypass the first assembly block.","subject":{"id":"f0e47ca8-1073-5e0a-b77f-c881660ee6d0","slug":"cpmp","display_name":"Cyclic pyranopterin monophosphate / cPMP","entity_type_key":"small_molecule"},"object":{"id":"c90c9196-a0ea-5a57-93b1-a897bdfe4ca4","slug":"mocd-biomarker-pattern","display_name":"Combined sulfur and purine biomarker pattern in Moco deficiency","entity_type_key":"cellular_process"},"evidence_count":1,"mechanism_event":{"id":"90265e41-9483-5a09-9ccd-d78554747545","stable_key":"dc8975b1-95ff-5d9b-be17-a1c04610cca7:mo-cpmp-type-a-event","event_type":"observed_intervention","label":"Supplying the missing precursor can bypass the first assembly block.","description":"Urinary S-sulfocysteine, xanthine and urate approached normal within two days in all 11 MoCD-A patients receiving cPMP; eight rapidly improved clinically.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"f0e47ca8-1073-5e0a-b77f-c881660ee6d0","slug":"cpmp","display_name":"Cyclic pyranopterin monophosphate / cPMP","entity_type_key":"small_molecule"},"role":"subject","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"c90c9196-a0ea-5a57-93b1-a897bdfe4ca4","slug":"mocd-biomarker-pattern","display_name":"Combined sulfur and purine biomarker pattern in Moco deficiency","entity_type_key":"cellular_process"},"role":"target","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""}]},"contexts":[{"dimension":"availability_state","value_text":"machinery_impairment","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null},{"dimension":"evidence_span","value_text":"{\"source_cache\": \"artifacts/molybdenum-research/26343839.abstract.txt\", \"locator\": \"Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets\", \"file_sha256\": \"2ad559ea27a88eb8d55c30c517ca0ca53d01125912e43a330ed1290f37aff3ad\", \"start_char\": 0, \"end_char\": 2265, \"text_sha256\": \"2ad559ea27a88eb8d55c30c517ca0ca53d01125912e43a330ed1290f37aff3ad\"}","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"experimental_model","value_text":"Prospective compassionate-use observational cohort of 16 neonates","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"exposure","value_text":"cPMP replacement in 11 type A and five type B patients","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"Nonrandomized rare-disease cohort. 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(2015). https://pubmed.ncbi.nlm.nih.gov/26343839/ DOI: 10.1016/s0140-6736(15)00124-5","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"tissue_or_cell_type","value_text":"MoCD biochemical markers and neurological outcomes","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":"07e0dfde-ffc4-5b3d-83c7-2ffa2088615a","evidence_kind":"source_excerpt","locator":"Lines 1184-1195","start_line":1184,"end_line":1195,"excerpt":"### mo-cpmp-type-a\nUrinary S-sulfocysteine, xanthine and urate approached normal within two days in all 11 MoCD-A patients receiving cPMP; eight rapidly improved clinically.\nCondition category: machinery_impairment\nnutrient_topic: Molybdenum research collection; topical membership is not evidence of a direct dietary effect.\nplain_language: Supplying the missing precursor can bypass the first assembly block.\norganism: Homo sapiens\ntissue_or_cell_type: MoCD biochemical markers and neurological outcomes\nexperimental_model: Prospective compassionate-use observational cohort of 16 neonates\nlimitations: Nonrandomized rare-disease cohort. Precursor replacement bypasses a specific biosynthetic step; it is not ordinary mineral supplementation.\nexposure: cPMP replacement in 11 type A and five type B patients\nevidence_span: {\"source_cache\": \"artifacts/molybdenum-research/26343839.abstract.txt\", \"locator\": \"Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets\", \"file_sha256\": \"2ad559ea27a88eb8d55c30c517ca0ca53d01125912e43a330ed1290f37aff3ad\", \"start_char\": 0, \"end_char\": 2265, \"text_sha256\": \"2ad559ea27a88eb8d55c30c517ca0ca53d01125912e43a330ed1290f37aff3ad\"}\n[mo-p26343839] Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study. 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