Trapped Neutrophil Syndrome (TNS) in Border Collies

Quick Summary

Trapped neutrophil syndrome is an inherited neutropenia in Border Collies that compromises the immune system, leading to chronic infection in affected dogs.

Phenotype: Affected puppies suffer from chronic infections resulting from a compromised immune system, are often smaller than their littermates and have developmental delays. Additionally, a narrow elongated skull and slender extremities have been observed in some affected dogs. Affected individuals can have infections from as early as 6 weeks of age, while a few may have mild clinical signs that are not observed until age 2 or older.

Mode of Inheritance: Autosomal recessive

Alleles: N = Normal, TNS = Trapped neutrophil syndrome

Breeds appropriate for testing: Border Collie

Explanation of Results:

  • Dogs with N/N genotype will not have trapped neutrophil syndrome and cannot transmit this variant to their offspring.
  • Dogs with N/TNS genotype will not be affected by trapped neutrophil syndrome, but are carriers. They may transmit this variant to 50% of their offspring. Matings between two carriers are predicted to produce 25% trapped neutrophil syndrome-affected puppies.
  • Dogs with TNS/TNS genotype will have trapped neutrophil syndrome.

Results of this test can be submitted to the OFA (Orthopedic Foundation for Animals)

Price

$50 one test per animal
$30 as additional test (same animal)

Panels Available
Additional Details

An inherited neutropenia (decrease in white blood cells) has been identified in Border Collies. This disease, called trapped neutrophil syndrome (TNS), has been found to be caused by a mutation in the Vesicle Protein Sorting 13B (VPS13B) gene. Affected puppies suffer from chronic infections resulting from a compromised immune system, are often smaller than their littermates and have developmental delays. Additionally, a narrow elongated skull and slender extremities have been observed in some affected dogs. Affected individuals can have infections from as early as 6 weeks of age, while a few may have mild clinical signs that are not observed until age 2 or older. TNS is inherited as an autosomal recessive disease, thus both sexes are equally affected and 2 copies of the defective allele are required to produce the disease. The defective allele has been observed at a rate of 7-8% worldwide in Border Collies.

 

Note: This test is specific for the deletion in the VPS13B gene associated with TNS in Border Collies.
Turnaround Time
3-6 business days

Species

Dog

Type of Test

Results Reported As
Test Result Trapped Neutrophil Syndrome
N/N No copies of TNS mutation. Dog is normal.
N/TNS 1 copy of TNS mutation. Dog is normal but is a carrier. Breedings between carriers are expected to produce 25% affected offspring.
TNS/TNS 2 copies of TNS mutation. Dog is affected.
References

Shearman, J.R., & Wilton, A.N. (2011). A canine model of Cohen syndrome: Trapped Neutrophil Syndrome. BMC Genomics 12:258. doi: 10.1186/1471-2164-12-258