API

Relationship: 9

Title

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Decreased, 3-hydroxyacyl-CoA dehydrogenase type-2 activity leads to Decreased, Mitochondrial fatty acid beta-oxidation

Upstream event

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Decreased, 3-hydroxyacyl-CoA dehydrogenase type-2 activity

Downstream event

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Decreased, Mitochondrial fatty acid beta-oxidation

Key Event Relationship Overview

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AOPs Referencing Relationship

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AOP Name Adjacency Weight of Evidence Quantitative Understanding
Peroxisomal Fatty Acid Beta-Oxidation Inhibition Leading to Steatosis adjacent High

Taxonomic Applicability

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Sex Applicability

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Life Stage Applicability

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Key Event Relationship Description

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Evidence Supporting this KER

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Biological Plausibility

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Empirical Evidence

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  • People with the G1528C mutation in HSD17B10 gene exhibit hepatomegaly and steatosis [1].
  • 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 [2].

Uncertainties and Inconsistencies

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Quantitative Understanding of the Linkage

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Response-response Relationship

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Time-scale

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Known modulating factors

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Known Feedforward/Feedback loops influencing this KER

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Domain of Applicability

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References

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  1. 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.
  2. 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.