Decreased, 3-hydroxyacyl-CoA dehydrogenase type-2 activity leads to Decreased, Mitochondrial fatty acid beta-oxidation
Upstream eventDecreased, 3-hydroxyacyl-CoA dehydrogenase type-2 activity
Decreased, Mitochondrial fatty acid beta-oxidation
Key Event Relationship Overview
AOPs Referencing Relationship
|AOP Name||Adjacency||Weight of Evidence||Quantitative Understanding|
|Peroxisomal Fatty Acid Beta-Oxidation Inhibition Leading to Steatosis||adjacent||High|
Life Stage Applicability
Key Event Relationship Description
Evidence Supporting this KER
- People with the G1528C mutation in HSD17B10 gene exhibit hepatomegaly and steatosis .
- Post mortem analyses of livers from children who died prior to 1 year of age demonstrated a link between steatosis \and 3-hydroxyacyl-CoA dehydrogenase activity .
Uncertainties and Inconsistencies
Quantitative Understanding of the Linkage
Known modulating factors
Known Feedforward/Feedback loops influencing this KER
Domain of Applicability
- Tyni T1, Rapola J, Paetau A, Palotie A, Pihko H. 1997. Pathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation. Pediatr Pathol Lab Med 17(3):427-47.
- Boles RG1, Buck EA, Blitzer MG, Platt MS, Cowan TM, Martin SK, Yoon H, Madsen JA, Reyes-Mugica M, Rinaldo P. 1998. Retrospective biochemical screening of fatty acid oxidation disorders in postmortem livers of 418 cases of sudden death in the first year of life. J Pediatr 132(6):924-33.