{"id":"89ecc7bb-0f06-5fb6-b479-93ae18f019e6","stable_key":"3b5aff9b-4086-5574-bfb4-3ea49ba520d7:coq10-quinone-rescue-assay","predicate":"restores_in_assay","statement":"Adding quinone restored CoQ-dependent respiratory activities in the tested deficient preparations.","claim_class":"mechanistic","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"ce2c7d6c-5b8f-578e-87d9-e6a080edb48f","mechanism_event_label":"An intact respiratory enzyme can still fail when its mobile electron carrier is missing.","subject":{"id":"ae9544c3-df78-54f8-80e7-5b643e5ba1cf","slug":"ubiquinone","display_name":"Ubiquinone","entity_type_key":"small_molecule"},"object":{"id":"cbae802f-75e0-5729-8306-2f7cb4ca6ce6","slug":"mitochondrial-electron-transfer","display_name":"Mitochondrial respiratory electron transfer","entity_type_key":"cellular_process"},"evidence_count":1,"mechanism_event":{"id":"ce2c7d6c-5b8f-578e-87d9-e6a080edb48f","stable_key":"3b5aff9b-4086-5574-bfb4-3ea49ba520d7:coq10-quinone-rescue-assay-event","event_type":"biochemical_relationship","label":"An intact respiratory enzyme can still fail when its mobile electron carrier is missing.","description":"Adding quinone restored CoQ-dependent respiratory activities in the tested deficient preparations.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"ae9544c3-df78-54f8-80e7-5b643e5ba1cf","slug":"ubiquinone","display_name":"Ubiquinone","entity_type_key":"small_molecule"},"role":"subject","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"cbae802f-75e0-5729-8306-2f7cb4ca6ce6","slug":"mitochondrial-electron-transfer","display_name":"Mitochondrial respiratory electron transfer","entity_type_key":"cellular_process"},"role":"target","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""}]},"contexts":[{"dimension":"evidence_span","value_text":"{\"source_cache\": \"artifacts/coq10-research/17332895.abstract.txt\", \"locator\": \"Primary indexed abstract; zero-based, end-exclusive Unicode character offsets\", \"file_sha256\": \"0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87\", \"start_char\": 0, \"end_char\": 1416, \"text_sha256\": \"0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87\"}","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"experimental_model","value_text":"Pedigrees, respiratory assays and yeast complementation","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"exposure","value_text":"PDSS1 D308E or COQ2 frameshift variants","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"Distinct families and mutations; quinone rescue in an assay is not equivalent to proven oral treatment of every organ.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"nutrient_topic","value_text":"Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect.","comparator":null,"unit":null,"notes":"","entity":{"slug":"coq10","display_name":"Coenzyme Q10 / CoQ10 redox system","entity_type_key":"chemical_species"}},{"dimension":"organism","value_text":"Human families and yeast validation","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"plain_language","value_text":"An intact respiratory enzyme can still fail when its mobile electron carrier is missing.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"[coq10-p17332895] Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders. (2007). https://pubmed.ncbi.nlm.nih.gov/17332895/ DOI: 10.1172/jci29089","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"tissue_or_cell_type","value_text":"CoQ-dependent respiratory function","comparator":null,"unit":null,"notes":"","entity":null}],"evidence":[{"id":"6d299923-97f9-5b74-b99c-0b6abe1f32a3","evidence_kind":"source_excerpt","locator":"Lines 216-227","start_line":216,"end_line":227,"excerpt":"### coq10-quinone-rescue-assay\nAdding quinone restored CoQ-dependent respiratory activities in the tested deficient preparations.\nCondition category: normal\nnutrient_topic: Coenzyme Q10 research collection; topical membership is not evidence of a direct dietary effect.\nplain_language: An intact respiratory enzyme can still fail when its mobile electron carrier is missing.\norganism: Human families and yeast validation\ntissue_or_cell_type: CoQ-dependent respiratory function\nexperimental_model: Pedigrees, respiratory assays and yeast complementation\nlimitations: Distinct families and mutations; quinone rescue in an assay is not equivalent to proven oral treatment of every organ.\nexposure: PDSS1 D308E or COQ2 frameshift variants\nevidence_span: {\"source_cache\": \"artifacts/coq10-research/17332895.abstract.txt\", \"locator\": \"Primary indexed abstract; zero-based, end-exclusive Unicode character offsets\", \"file_sha256\": \"0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87\", \"start_char\": 0, \"end_char\": 1416, \"text_sha256\": \"0a500ba6d7c7b5f32817074367d71993504fbd9c5a10cb76038faf718fb79b87\"}\n[coq10-p17332895] Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders. 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