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Laryngeal paralysis (LP) of the Bull terrier

Neurological · Dog

Early-onset laryngeal paralysis in the Bull terrier and Miniature Bull terrier in which an insertion in the RAPGEF6 gene acts as a major risk factor. It is a multifactorial trait: the variant modulates risk but does not determine it, so homozygosity should be interpreted as increased risk and not as a diagnosis of disease.
Inheritance patternMultifactorial (OMIA:002222-9615); recessive risk allele with incomplete penetrance
Gene / MutationRAPGEF6 c.1793_1794ins36 (36-bp insertion in exon 15)
PenetranceIncomplete. Homozygosity confers a 10- to 17-fold increased risk (Hadji Rasouliha et al. 2019), but not all homozygotes develop laryngeal paralysis.
Codelptb
Turnaround time7 days
Price41,60 €

Incidence

The allele was found in Bull terrier and Miniature Bull terrier and was absent in more than 1000 controls of other breeds (Hadji Rasouliha et al. 2019); no published population prevalence.

Breeder management

- Do not interpret homozygosity as a diagnosis: it is a risk factor, not deterministic
- It can be used as a selection tool in affected lines
- Combine with clinical assessment of the airway and family history
- Avoid presenting it as a diagnostic test or sole exclusion criterion
- Inform about its multifactorial nature

Specialist notes

Differential diagnosis with other hereditary laryngeal paralyses/polyneuropathies (RAB3GAP1, CNTNAP1, ARHGEF10, GJA9, SBF2). Because of its multifactorial nature, integrate the result with the clinical phenotype.

References

Hadji Rasouliha S et al. 2019. A RAPGEF6 variant constitutes a major risk factor for laryngeal paralysis in dogs. PLoS Genet. PMID: 31647804

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