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Reverse query / human → dog

developmental and epileptic encephalopathy, 39.

A rare mitochondrial substrate carrier disorder characterized by severe muscular hypotonia, seizures (with or without episodic apnea) beginning in the first year of life, and arrested psychomotor development (affecting mainly motor skills). Severe spasticity with hyperreflexia has also been reported. Global cerebral hypomyelination is a characteristic imaging feature of this disease.

Which dogs are a natural model of this human disease. Each row is a distinct gene pathway with a canine model, ranked by evidence strength. We assert the canine disease models the human one (gene-level), never that a dog allele equals a human variant.

1 model pathway 1 OMIA-anchored MONDO:0013056 ↗ OMIM 612949 ↗
Canine model pathway Evidence Ortholog Human anchor Canine variant · assembly
SLC25A12 → SLC25A12 OMIA model-of OMIA-anchored one_to_one gene-level (no single variant)
The boundary of this model. The rows above are the characterized pathways, human genes of this disease with a canine model in our substrate. We do not hold a GenCC established-gene panel for this specific Mondo id, so the full gene landscape is not enumerated here; we show the canine models we hold and do not imply full coverage.
A candidate model is a computational hypothesis (gene-level model-of), never a confirmed model; confirmation is DNA plus phenotype, in the lab. Canine coordinates are on UU_Cfam_GSD_1.0 and carry their assembly (no cross-assembly comparison without a scored liftover). Sources: OMIA, ClinVar (Landrum 2018), Ensembl Compara orthology, Mondo. Ranked by evidence strength within this human disease.