{"id":"99dc5f5c-cffb-5c86-85b0-2da0c42049bb","stable_key":"dc8975b1-95ff-5d9b-be17-a1c04610cca7:mo-cpmp-type-b","predicate":"did_not_restore_in_type_b","statement":"The five MoCD-B patients showed no biochemical or clinical response to cPMP.","claim_class":"observational","status":"source_derived_draft","evidence_grade":"ungraded","direction":"neutral","is_public":true,"mechanism_event_id":"b640ba63-f4ab-5415-842c-220e5e584599","mechanism_event_label":"Supplying an upstream precursor cannot repair a broken downstream step.","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":"b640ba63-f4ab-5415-842c-220e5e584599","stable_key":"dc8975b1-95ff-5d9b-be17-a1c04610cca7:mo-cpmp-type-b-event","event_type":"observed_intervention","label":"Supplying an upstream precursor cannot repair a broken downstream step.","description":"The five MoCD-B patients showed no biochemical or clinical response to cPMP.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"cff081bb-6401-5e3e-965c-a32e8894da08","slug":"mocs2-mpt-synthase","display_name":"Human MOCS2A-MOCS2B molybdopterin synthase","entity_type_key":"protein_complex"},"role":"downstream machinery","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"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":1,"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":2,"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. Precursor replacement bypasses a specific biosynthetic step; it is not ordinary mineral supplementation.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"nutrient_topic","value_text":"Molybdenum research collection; topical membership is not evidence of a direct dietary effect.","comparator":null,"unit":null,"notes":"","entity":{"slug":"molybdenum","display_name":"Molybdenum","entity_type_key":"nutrient_element"}},{"dimension":"organism","value_text":"Homo sapiens","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"plain_language","value_text":"Supplying an upstream precursor cannot repair a broken downstream step.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"[mo-p26343839] Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study. (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":"5b3e2c44-22e8-56b5-846a-07823c8af19a","evidence_kind":"source_excerpt","locator":"Lines 1197-1208","start_line":1197,"end_line":1208,"excerpt":"### mo-cpmp-type-b\nThe five MoCD-B patients showed no biochemical or clinical response to cPMP.\nCondition category: machinery_impairment\nnutrient_topic: Molybdenum research collection; topical membership is not evidence of a direct dietary effect.\nplain_language: Supplying an upstream precursor cannot repair a broken downstream step.\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. (2015). https://pubmed.ncbi.nlm.nih.gov/26343839/ DOI: 10.1016/s0140-6736(15)00124-5","model_system":"Prospective compassionate-use observational cohort of 16 neonates","directness":"author_interpretation","verification_status":"source_derived_draft","notes":"Exact curation-document quotation, not publisher quotation. Study references: [mo-p26343839] Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study. (2015). https://pubmed.ncbi.nlm.nih.gov/26343839/ DOI: 10.1016/s0140-6736(15)00124-5","relationship":"supports","weight":1.0,"link_notes":"","source":{"id":"1aa6da60-8284-5252-8dd9-ac2250d5ced5","stable_key":"import-dc8975b1-95ff-5d9b-be17-a1c04610cca7","title":"Molybdenum: cofactor assembly, sulfur metabolism and nutrient interactions (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":"143147eb9ac7fe750bdd33bb50d7773bc0cd49cc94faf219aebce3c3d760dd8f","revision_id":"90c2239d-290b-5e16-815a-f94d30569ebd","review_status":"unverified_draft","notes":""}}],"relations":[],"conflicts":[],"corrections":[],"research":null}