Other names: PLN, Glomerulopathy
Affected genes: KIRREL2, NPHS1
Transmission: Complex
Mutations: KIRREL2: CanFam3.1 1g.116785027 C>G; NPHS1: CanFam3.1 1g.116806124 G>A
OMIA link: 001326-9615

Appropriate breeds for testing: Soft Coated Wheaten Terrier,  Airedale Terrier

Please note:
Protein-losing nephropathy is complexly inherited in dogs. For this test, two mutations are tested, one on the Kirrel2 gene and the other on the NPHS1 gene. If a dog inherits two copies of each mutation, it has a high risk of developing the disease. Dogs that inherit any other combination of mutations in KIRREL2 and NPHS1 are at unknown risk.

Symptoms:
Protein loss nephropathy is an inherited disease characterized by an abnormality of blood proteins being filtered by the kidneys and ending up lost in the urine. Usually, non-specific symptoms such as vomiting, diarrhea, excessive thirst and weight loss appear in adult dogs.

References:
Littman MP, Dambach DM, Vaden SL, Giger U. Familial protein-losing enteropathy and protein-losing nephropathy in Soft Coated Wheaten Terriers: 222 cases (1983-1997). J Vet Intern Med. 2000 Jan-Feb;14(1):68-80.
Littman MP, Wiley CA, Raducha MG, Henthorn PS. Glomerulopathy and mutations in NPHS1 and KIRREL2 in soft-coated Wheaten Terrier dogs. Mom Genome. 2013 Apr;24(3-4):119-26.