{"id":"8e6870b7-f717-51a5-aee9-44a075491405","stable_key":"dc8975b1-95ff-5d9b-be17-a1c04610cca7:mo-suox-cofactors","predicate":"forms_catalytic_site_of","statement":"SUOX sulfite oxidation depends on Moco at the catalytic site and a separate heme cofactor.","claim_class":"mechanistic","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"2fe2862f-5735-5626-8dc9-9ddf826a345b","mechanism_event_label":"Molybdenum and iron-containing heme perform different jobs within this enzyme.","subject":{"id":"ac4bcc03-66fb-52c1-b191-9ecb2124659f","slug":"molybdenum-cofactor","display_name":"Molybdenum cofactor / Moco","entity_type_key":"small_molecule"},"object":{"id":"bf313203-7930-5d8b-9c8c-a10559dafcaf","slug":"suox","display_name":"Human sulfite oxidase / SUOX","entity_type_key":"protein"},"evidence_count":1,"mechanism_event":{"id":"2fe2862f-5735-5626-8dc9-9ddf826a345b","stable_key":"dc8975b1-95ff-5d9b-be17-a1c04610cca7:mo-suox-cofactors-event","event_type":"biochemical_relationship","label":"Molybdenum and iron-containing heme perform different jobs within this enzyme.","description":"SUOX sulfite oxidation depends on Moco at the catalytic site and a separate heme cofactor.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"2e1f7e0a-8b54-5ea3-9d34-01e7167f9097","slug":"heme","display_name":"Heme","entity_type_key":"small_molecule"},"role":"cofactor","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"89bcaf42-b4ab-5760-8c2e-44eace10cee0","slug":"iron","display_name":"Iron","entity_type_key":"nutrient_element"},"role":"heme constituent","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""},{"entity":{"id":"ac4bcc03-66fb-52c1-b191-9ecb2124659f","slug":"molybdenum-cofactor","display_name":"Molybdenum cofactor / Moco","entity_type_key":"small_molecule"},"role":"subject","stoichiometry":null,"state_label":"","sequence_order":2,"notes":""},{"entity":{"id":"bf313203-7930-5d8b-9c8c-a10559dafcaf","slug":"suox","display_name":"Human sulfite oxidase / SUOX","entity_type_key":"protein"},"role":"target","stoichiometry":null,"state_label":"","sequence_order":3,"notes":""}]},"contexts":[{"dimension":"evidence_span","value_text":"{\"source_cache\": \"artifacts/molybdenum-research/31127934.abstract.txt\", \"locator\": \"Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets\", \"file_sha256\": \"d0efd64e9cde371d0223d63607f5f6b76aada3a628c526f915d9b306c9bef7de\", \"start_char\": 0, \"end_char\": 1649, \"text_sha256\": \"d0efd64e9cde371d0223d63607f5f6b76aada3a628c526f915d9b306c9bef7de\"}","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"experimental_model","value_text":"Human SUOX Gly362Ser patient fibroblasts and recombinant enzyme maturation assays","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"exposure","value_text":"G362S versus WT protein; Moco reconstitution and molybdate supplementation in culture","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"One genotype; in-vitro molybdate rescue is not demonstrated clinical treatment for all SUOX defects.","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":"Molybdenum and iron-containing heme perform different jobs within this enzyme.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"[mo-p31127934] Impaired mitochondrial maturation of sulfite oxidase in a patient with severe sulfite oxidase deficiency. (2019). https://pubmed.ncbi.nlm.nih.gov/31127934/ DOI: 10.1093/hmg/ddz109","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"tissue_or_cell_type","value_text":"Mitochondrial intermembrane space; patient fibroblasts","comparator":null,"unit":null,"notes":"","entity":null}],"evidence":[{"id":"14a119ba-4978-5738-be06-e758a92513eb","evidence_kind":"source_excerpt","locator":"Lines 599-610","start_line":599,"end_line":610,"excerpt":"### mo-suox-cofactors\nSUOX sulfite oxidation depends on Moco at the catalytic site and a separate heme cofactor.\nCondition category: normal\nnutrient_topic: Molybdenum research collection; topical membership is not evidence of a direct dietary effect.\nplain_language: Molybdenum and iron-containing heme perform different jobs within this enzyme.\norganism: Homo sapiens\ntissue_or_cell_type: Mitochondrial intermembrane space; patient fibroblasts\nexperimental_model: Human SUOX Gly362Ser patient fibroblasts and recombinant enzyme maturation assays\nlimitations: One genotype; in-vitro molybdate rescue is not demonstrated clinical treatment for all SUOX defects.\nexposure: G362S versus WT protein; Moco reconstitution and molybdate supplementation in culture\nevidence_span: {\"source_cache\": \"artifacts/molybdenum-research/31127934.abstract.txt\", \"locator\": \"Exact primary indexed abstract; zero-based, end-exclusive Unicode character offsets\", \"file_sha256\": \"d0efd64e9cde371d0223d63607f5f6b76aada3a628c526f915d9b306c9bef7de\", \"start_char\": 0, \"end_char\": 1649, \"text_sha256\": \"d0efd64e9cde371d0223d63607f5f6b76aada3a628c526f915d9b306c9bef7de\"}\n[mo-p31127934] Impaired mitochondrial maturation of sulfite oxidase in a patient with severe sulfite oxidase deficiency. 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