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Fox Terrier pack: Spinocerebellar ataxia (SCA), Primary lens luxation (PLL) and van den Ende-Gupta syndrome (VDEGS)
General · Dog
Multi-disease genetic panel for the Fox Terrier grouping three molecular tests for hereditary conditions described in the breed: spinocerebellar ataxia (SCA), primary lens luxation (PLL) and van den Ende-Gupta syndrome (VDEGS). Each condition has its own molecular basis and inheritance. The panel is complementary to ocular and neurological examination in breeding selection.
Incidence
Applicable breed: Fox Terrier (smooth and wire-haired depending on the condition). Reliable carrier frequencies in the breeding population are not published systematically (limited data); PLL is relatively frequent in small terrier lines.
Clinical signs
- Progressive ataxia, tremor and cerebellar signs from a young age (SCA)\n- Lethargy, failure to thrive, depigmented hair and goitre from puppyhood (CHG)\n- Altered vision, painful red eye and mydriasis (acute PLL)\n- Lens luxation with secondary glaucoma and blindness (PLL)\n- Skeletal malformations: limb bowing, prognathism and facial abnormalities (VDEGS)\n- Respiratory and swallowing difficulty in some cases (VDEGS)
History
Primary lens luxation was linked to ADAMTS17 from the work of Sargan, Gould and colleagues in terriers and small breeds. Van den Ende-Gupta syndrome has been described in the Fox Terrier as a recessive congenital bone entity; the exact molecular assignment in the accessible literature is less solid and must be verified in the primary source. Hereditary spinocerebellar ataxia of the Fox Terrier has been described as a breed-specific entity; the causal gene is not firmly established in the accessible literature (in related terrier breeds it has been associated with KCNJ10, but transfer to the Fox Terrier must be verified).
Breeder management
- Genotype breeding animals before mating; the panel covers three conditions in a single sample\n- For SCA and VDEGS (recessive): do not mate two carriers — 25% risk of affected homozygotes; carrier×clear is safe for offspring intended for breeding if tested\n- For PLL (incomplete dominant): heterozygotes can transmit the variant to 50% of the offspring; prioritise clear animals for breeding and carry out an annual ophthalmological assessment\n- While the SCA and VDEGS genes are being confirmed, avoid mating affected animals and keep a genealogical record of lines with cases\n- After a confirmed clinical case, do not repeat the parental mating and communicate the status to the buyer
Specialist notes
SCA must be differentiated from other cerebellar ataxias and from acquired causes (infectious, toxic, neoplastic). PLL must be differentiated from traumatic luxation or luxation secondary to uveitis. VDEGS has a differential diagnosis with other congenital skeletal dysplasias; molecular study confirms it. Annual ocular examination (ECVO/CERF) in all breeding animals. | NOTE 2026-09-18: CHG/TPO in the Fox Terrier with no published cases (limited data).
References
3. Gould D y cols. ADAMTS17 y luxación primaria de lente (PMID 22050825)
Tests included in this pack (3)
Price: 121,13 € · Turnaround time: 15 days