{"id":"09f44ed4-ccd7-5853-9434-0c6c965a148b","stable_key":"1310afbd-6010-586e-805d-551d846da421:b6-hpp-enzyme-replacement-ratio","predicate":"replacement_associated_with_lower_plp_pl_ratio","statement":"Enzyme replacement was associated with lower serum PLP: PL ratios; CSF PL was higher in treated than untreated cases.","claim_class":"observational","status":"source_derived_draft","evidence_grade":"ungraded","direction":"negative","is_public":true,"mechanism_event_id":"9ed2ca56-294e-55ab-a603-66290c2fda0d","mechanism_event_label":"Restoring enzyme activity changed B6 handling markers, including the sampled fluid around the brain.","subject":{"id":"3148c377-9b4c-56ca-bc36-081bf1905d57","slug":"alpl","display_name":"Tissue-nonspecific alkaline phosphatase ALPL / TNAP","entity_type_key":"protein"},"object":{"id":"149919aa-d520-51b4-85d6-5a6f534ee8a3","slug":"serum-plp-to-pl-ratio","display_name":"Serum PLP to pyridoxal ratio","entity_type_key":"cellular_process"},"evidence_count":1,"mechanism_event":{"id":"9ed2ca56-294e-55ab-a603-66290c2fda0d","stable_key":"1310afbd-6010-586e-805d-551d846da421:b6-hpp-enzyme-replacement-ratio-event","event_type":"observed_intervention","label":"Restoring enzyme activity changed B6 handling markers, including the sampled fluid around the brain.","description":"Enzyme replacement was associated with lower serum PLP: PL ratios; CSF PL was higher in treated than untreated cases.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"9bf6269d-9e01-553d-bd8b-11a0d613bfca","slug":"pyridoxal","display_name":"Pyridoxal","entity_type_key":"small_molecule"},"role":"csf_vitamer","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"3148c377-9b4c-56ca-bc36-081bf1905d57","slug":"alpl","display_name":"Tissue-nonspecific alkaline phosphatase ALPL / TNAP","entity_type_key":"protein"},"role":"subject","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""},{"entity":{"id":"149919aa-d520-51b4-85d6-5a6f534ee8a3","slug":"serum-plp-to-pl-ratio","display_name":"Serum PLP to pyridoxal ratio","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":"experimental_model","value_text":"Over 100 hypophosphatasia cases, serum vitamers and available CSF; supplementation/enzyme-replacement subgroups.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"exposure","value_text":"Observational clinical sampling by subtype and treatment; no randomized B6 efficacy comparison.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"Nonrandomized treatment comparisons; does not directly measure transport flux or establish neurological benefit.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"nutrient_topic","value_text":"Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect.","comparator":null,"unit":null,"notes":"","entity":{"slug":"vitamin-b6","display_name":"Vitamin B6","entity_type_key":"chemical_species"}},{"dimension":"organism","value_text":"Homo sapiens","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"plain_language","value_text":"Restoring enzyme activity changed B6 handling markers, including the sampled fluid around the brain.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"[b6-akitomo2025] Vitamin B6 Status in Hypophosphatasia: Association With Clinical Severity, Diagnostic Utility, and Effects on Vitamin B6 Metabolism by Supplementation and Enzyme Replacement Therapy (2025). https://pubmed.ncbi.nlm.nih.gov/40387451/ DOI: 10.1002/jimd.70036","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"tissue_or_cell_type","value_text":"Serum and cerebrospinal fluid","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":"4547c1e2-49d3-5f80-acdb-eaba7b3afec1","evidence_kind":"source_excerpt","locator":"Lines 1657-1667","start_line":1657,"end_line":1667,"excerpt":"### b6-hpp-enzyme-replacement-ratio\nEnzyme replacement was associated with lower serum PLP: PL ratios; CSF PL was higher in treated than untreated cases.\nCondition category: machinery_impairment\nnutrient_topic: Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect.\nplain_language: Restoring enzyme activity changed B6 handling markers, including the sampled fluid around the brain.\norganism: Homo sapiens\ntissue_or_cell_type: Serum and cerebrospinal fluid\nexperimental_model: Over 100 hypophosphatasia cases, serum vitamers and available CSF; supplementation/enzyme-replacement subgroups.\nlimitations: Nonrandomized treatment comparisons; does not directly measure transport flux or establish neurological benefit.\nexposure: Observational clinical sampling by subtype and treatment; no randomized B6 efficacy comparison.\n[b6-akitomo2025] Vitamin B6 Status in Hypophosphatasia: Association With Clinical Severity, Diagnostic Utility, and Effects on Vitamin B6 Metabolism by Supplementation and Enzyme Replacement Therapy (2025). https://pubmed.ncbi.nlm.nih.gov/40387451/ DOI: 10.1002/jimd.70036","model_system":"Over 100 hypophosphatasia cases, serum vitamers and available CSF; supplementation/enzyme-replacement subgroups.","directness":"author_interpretation","verification_status":"source_derived_draft","notes":"Exact curation-document quotation, not publisher quotation. Study references: [b6-akitomo2025] Vitamin B6 Status in Hypophosphatasia: Association With Clinical Severity, Diagnostic Utility, and Effects on Vitamin B6 Metabolism by Supplementation and Enzyme Replacement Therapy (2025). https://pubmed.ncbi.nlm.nih.gov/40387451/ DOI: 10.1002/jimd.70036","relationship":"supports","weight":1.0,"link_notes":"","source":{"id":"251773bb-16f5-5903-b135-db4a61d9dec4","stable_key":"import-1310afbd-6010-586e-805d-551d846da421","title":"Vitamin B6: mechanisms, deficiency 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":"ef0019b344b2219220f801a84d0d138ff6880c1be1a59540d9034bfa4334f61e","revision_id":"cac3555f-48af-5c52-a84e-add4482c87fb","review_status":"unverified_draft","notes":""}}],"relations":[],"conflicts":[],"corrections":[],"research":null}