{"id":"e0053fa6-4e0f-5e93-97d0-97e531bb5e58","stable_key":"1310afbd-6010-586e-805d-551d846da421:b6-hpp-severity-vitamer-pattern","predicate":"impairment_severity_associated_with_vitamer_pattern","statement":"In the 2025 cohort, untreated HPP had higher serum PLP and PLP: PL ratios; severe perinatal disease had lower PL than benign perinatal disease and the lowest PA across subtypes.","claim_class":"observational","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"9b187773-c354-520c-84f7-0d203aaa49f1","mechanism_event_label":"Disease severity changes which B6 forms accumulate or become low.","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":"9b187773-c354-520c-84f7-0d203aaa49f1","stable_key":"1310afbd-6010-586e-805d-551d846da421:b6-hpp-severity-vitamer-pattern-event","event_type":"observed_intervention","label":"Disease severity changes which B6 forms accumulate or become low.","description":"In the 2025 cohort, untreated HPP had higher serum PLP and PLP: PL ratios; severe perinatal disease had lower PL than benign perinatal disease and the lowest PA across subtypes.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"9bf6269d-9e01-553d-bd8b-11a0d613bfca","slug":"pyridoxal","display_name":"Pyridoxal","entity_type_key":"small_molecule"},"role":"vitamer","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"befbcf98-c6a9-5b72-a039-2d6b9f3bde12","slug":"4-pyridoxic-acid","display_name":"4-Pyridoxic acid","entity_type_key":"small_molecule"},"role":"catabolite","stoichiometry":null,"state_label":"","sequence_order":1,"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":2,"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":3,"notes":""}]},"contexts":[{"dimension":"availability_state","value_text":"biomarker_context","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":"Observational subtype comparison; contextual difference from broader pediatric cohorts, not an editorial conflict.","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":"Disease severity changes which B6 forms accumulate or become low.","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":"Human blood and whole-body measurements","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"trigger_kind","value_text":"biomarker_context","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null}],"evidence":[{"id":"09a0f401-3f91-5bb7-ae13-8a12c12ea513","evidence_kind":"source_excerpt","locator":"Lines 1645-1655","start_line":1645,"end_line":1655,"excerpt":"### b6-hpp-severity-vitamer-pattern\nIn the 2025 cohort, untreated HPP had higher serum PLP and PLP: PL ratios; severe perinatal disease had lower PL than benign perinatal disease and the lowest PA across subtypes.\nCondition category: biomarker_context\nnutrient_topic: Vitamin B6 research collection; topical membership is not evidence of a direct dietary effect.\nplain_language: Disease severity changes which B6 forms accumulate or become low.\norganism: Homo sapiens\ntissue_or_cell_type: Human blood and whole-body measurements\nexperimental_model: Over 100 hypophosphatasia cases, serum vitamers and available CSF; supplementation/enzyme-replacement subgroups.\nlimitations: Observational subtype comparison; contextual difference from broader pediatric cohorts, not an editorial conflict.\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}