{"id":"0262f56d-b771-5c59-a940-4b54bfa2e31b","stable_key":"c3df3634-4c3a-5099-a5d9-e4f6344c1084:aass-sdh-saccharopine-accumulation","predicate":"selective_impairment_accumulates","statement":"Selective AASS saccharopine-dehydrogenase impairment while reductase activity persists causes saccharopine accumulation.","claim_class":"mechanistic","status":"source_derived_draft","evidence_grade":"ungraded","direction":"context_dependent","is_public":true,"mechanism_event_id":"2b207550-7214-54cf-8ea9-7cf9c39055b6","mechanism_event_label":"Saccharopine builds up when its production continues but its removal fails.","subject":{"id":"3e38b3e2-0dad-59ef-81d9-6c2af7e22d73","slug":"aass","display_name":"AASS","entity_type_key":"protein"},"object":{"id":"d70bb0d3-bb08-5b47-9ac0-5c7d8b56dd24","slug":"saccharopine","display_name":"Saccharopine","entity_type_key":"small_molecule"},"evidence_count":1,"mechanism_event":{"id":"2b207550-7214-54cf-8ea9-7cf9c39055b6","stable_key":"c3df3634-4c3a-5099-a5d9-e4f6344c1084:aass-sdh-saccharopine-accumulation-event","event_type":"biochemical_relationship","label":"Saccharopine builds up when its production continues but its removal fails.","description":"Selective AASS saccharopine-dehydrogenase impairment while reductase activity persists causes saccharopine accumulation.","status":"provisional","compartment":null,"participants":[{"entity":{"id":"3e38b3e2-0dad-59ef-81d9-6c2af7e22d73","slug":"aass","display_name":"AASS","entity_type_key":"protein"},"role":"impaired_machinery","stoichiometry":null,"state_label":"","sequence_order":0,"notes":""},{"entity":{"id":"d70bb0d3-bb08-5b47-9ac0-5c7d8b56dd24","slug":"saccharopine","display_name":"Saccharopine","entity_type_key":"small_molecule"},"role":"accumulating_metabolite","stoichiometry":null,"state_label":"","sequence_order":1,"notes":""},{"entity":{"id":"5fff6664-209e-5c35-b88e-ac15c1c5fbc0","slug":"l-lysine","display_name":"L-Lysine","entity_type_key":"small_molecule"},"role":"upstream_substrate","stoichiometry":null,"state_label":"","sequence_order":2,"notes":""}]},"contexts":[{"dimension":"affected_machinery","value_text":"AASS saccharopine dehydrogenase","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"availability_state","value_text":"machinery_impairment","comparator":null,"unit":null,"notes":"Imported condition classification; unverified.","entity":null},{"dimension":"deficiency_not_equivalent","value_text":"Dietary lysine deficiency or complete AASS knockout","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"experimental_model","value_text":"AASS-domain mutant worms and engineered mice","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"limitations","value_text":"Selective downstream-domain failure differs from complete AASS loss.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"organism","value_text":"Caenorhabditis elegans and Mus musculus","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"plain_language","value_text":"Saccharopine builds up when its production continues but its removal fails.","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"primary_references","value_text":"[zhou2019] The lysine catabolite saccharopine impairs development by disrupting mitochondrial homeostasis (2019). https://pmc.ncbi.nlm.nih.gov/articles/PMC6363459/ DOI: 10.1083/jcb.201807204","comparator":null,"unit":null,"notes":"","entity":null},{"dimension":"tissue_or_cell_type","value_text":"Worm hypodermis and mouse liver","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":"f370bf0c-b7d7-5f39-ab0e-967dfe01552f","evidence_kind":"source_excerpt","locator":"Lines 230-240","start_line":230,"end_line":240,"excerpt":"### aass-sdh-saccharopine-accumulation\nSelective AASS saccharopine-dehydrogenase impairment while reductase activity persists causes saccharopine accumulation.\nPlain language: Saccharopine builds up when its production continues but its removal fails.\nCondition category: machinery_impairment\norganism: Caenorhabditis elegans and Mus musculus\ntissue_or_cell_type: Worm hypodermis and mouse liver\nexperimental_model: AASS-domain mutant worms and engineered mice\nlimitations: Selective downstream-domain failure differs from complete AASS loss.\naffected_machinery: AASS saccharopine dehydrogenase\ndeficiency_not_equivalent: Dietary lysine deficiency or complete AASS knockout\n[zhou2019] The lysine catabolite saccharopine impairs development by disrupting mitochondrial homeostasis (2019). https://pmc.ncbi.nlm.nih.gov/articles/PMC6363459/ DOI: 10.1083/jcb.201807204","model_system":"AASS-domain mutant worms and engineered mice","directness":"author_interpretation","verification_status":"source_derived_draft","notes":"Exact quote from the accompanying curation document, not from publisher text. Original study references: [zhou2019] The lysine catabolite saccharopine impairs development by disrupting mitochondrial homeostasis (2019). https://pmc.ncbi.nlm.nih.gov/articles/PMC6363459/ DOI: 10.1083/jcb.201807204","relationship":"supports","weight":1.0,"link_notes":"","source":{"id":"7633bde7-dcc9-5086-91c6-a45eb96857f3","stable_key":"import-c3df3634-4c3a-5099-a5d9-e4f6344c1084","title":"L-Lysine: mechanism-first literature curation (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":"93998d47c21525409ba82f1c82ededf2893dff15fbe61c7deffc175b0e298e97","revision_id":"3897e31f-6624-59e1-a053-8a81b7361632","review_status":"unverified_draft","notes":""}}],"relations":[],"conflicts":[],"corrections":[],"research":null}