Polyneuropathy (Leonberger Type 2, LPN2)

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Find out if your Leonberger could develop Polyneuropathy (Type 2) at CAGT.

Turnaround 1-2 weeks
Breed(s) , ,
OMIA OMIA002119-9615
Aliases , ,

Results from this test will be reported as detailed in the Registry Reporting list.


Part of the official UK Kennel Club testing scheme in Leonberger

Polyneuropathy Type 2 (LPN2) in Leonbergers is an inherited polyneuropathy (PN), which is characterised by generalised weakness, low muscle tone, exercise intolerance, gait abnormalities and muscle atrophy of the pelvic limbs. Degeneration of the recurrent laryngeal nerve results in laryngeal paralysis, which causes abnormal barking and breathing sounds. Age of onset ranges from less than one to 10 years of age, with an average age of six years. Dogs with at least on copy of the LPN2 variant explain 21% of all cases of PN in the breed.

Autosomal Dominant

The 2 bp deletion in the gene called GJA9 that causes Polyneuropathy in Leonberger is autosomal dominant, with incomplete penetrance. This means that dogs that carry one or two copies of the mutation (heterozygotes and homozygotes respectively) may develop Polyneuropathy during their lives, but not necessarily. They will also pass the mutation on to all (for homozygotes) or about half (for heterozygotes) of any offspring they have. Breeding dogs that will not develop Polyneuropathy should be the breeder’s priority, with a reduction in mutation frequency within the whole breed being the secondary, longer-term target.
Carriers should not be bred from, even if they are mated to a dog that has also been tested and is clear of the GJA9 mutation (i.e. carry no copies of the mutation). If a carrier is mated to a clear dog approximately half of the resulting puppies will also be carriers and will develop Polyneuropathy caused by the GJA9 mutation during their lifetime.

Gene GJA9
Variant c.1107_1108delAG
Assay Type Variant Specific
Inheritance Autosomal Dominant
Severity Moderate: Affected animals experience significant discomfort or dysfunction, management/treatment costs may be relatively high and life expectancy can be reduced.

Becker D, KM Minor, A Letko et al. (2017) A GJA9 frameshift variant is associated with polyneuropathy in Leonberger dogs. BMC Genomics. 18(1): 662. DOI: 10.1186/s12864-017-4081-z.