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Neuroaxonal dystrophy (NAD) in the Rottweiler

Neurological · Dog

A recessive neurodegenerative disease of the Rottweiler with onset in the young adult, with axonal spheroids mainly affecting sensory terminals of the central nervous system. It is associated with a missense mutation in the VPS11 gene. A test complementary to, not a substitute for, clinical examination.
Inheritance patternAutosomal recessive (OMIA; Lucot 2018). Affected homozygotes; asymptomatic carrier heterozygotes.
Gene / MutationVPS11 c.2504A>G p.(H835R) (OMIA:002152-9615, variant 995; g.14777774T>C, CanFam3.1).
PenetranceHigh in homozygotes according to the described cases; heterozygotes are asymptomatic carriers.
Codejpvb
Turnaround time15 days
Price52,60 €

Incidence

Rottweiler. Estimated allele frequency around 2.3% (Lucot 2018); limited data outside the breed.

Breeder management

- Genotype breeding animals before mating\n- Avoid carrier x carrier mating (25% affected)\n- A carrier may be mated to a clear animal: produces no affected offspring\n- Do not breed affected homozygotes

Specialist notes

Confirm the diagnosis with an antemortem genetic test. Include VPS11 in the differential diagnosis of unexplained neuroaxonal dystrophy cases and as a model for human VPS11 leukoencephalopathy.

References

1. Lucot KL et al. 2018, A missense mutation in the vacuolar protein sorting 11 (VPS11) gene is associated with neuroaxonal dystrophy in Rottweiler dogs. G3 (Bethesda). PMID: 29945969. OMIA:002152-9615.

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