The breed can be affected by a genetic disease known as Samoyed hereditary glomerulopathy, a kidney disease.
The disease is known to be caused by an X-linked dominant faulty allele and therefore the disease is more severe in male Samoyeds.
Also known as hereditary nephritis, it is caused by a nonsense mutation in codon 1027 of the COL4A5 gene on the X chromosome (glycine to stop codon), which is similar to Alport's syndrome in humans.

Carrier females do develop mild symptoms after 2–3 months of age, but mostly do not go on to develop kidney failure.
The disease is caused by a defect in the structure of the type-IV collagen fibrils of the glomerular basement membrane.
As a consequence, the collagen fibrils of the glomerular basement membrane are unable to form cross-links, so the structural integrity is weakened and the membrane is more susceptible to "wear-and-tear" damage.
As the structure of the basement membrane begins to degenerate, plasma proteins are lost in the urine and symptoms begin to appear.
Affected males appear healthy for the first three months of life, but then symptoms start to appear and worsen as the disease progresses: the dog becomes lethargic and muscle wastage occurs, as a result of proteinuria.
From three months of age onwards, a reduced glomerular filtration rate is detected, indicative of progressive kidney failure.

Clinically, proteinuria is found in both sexes from the age of three to four months; in dogs older than this, kidney failure in combination with more or less pronounced hearing loss occurs swiftly and death at the age of 8 to 15 months is expected.
In heterozygous females, the disease develops slowly.
The disease can be treated to slow down the development by use of cyclosporine A and ACE inhibitors, but not be stopped.

If a carrier female is mated with a healthy stud dog, the female offspring have a 50% chance of being carriers for the disease, and any male offspring have a 50% chance of being affected by the disease.
A genetic test is available for this disease.
The breed can be affected by a genetic disease known as Samoyed hereditary glomerulopathy, a kidney disease. The disease is known to be caused by an X-linked dominant faulty allele and therefore the disease is more severe in male Samoyeds. Also known as hereditary nephritis, it is caused by a nonsense mutation in codon 1027 of the COL4A5 gene on the X chromosome (glycine to stop codon), which is similar to Alport's syndrome in humans.
Carrier females do develop mild symptoms after 2–3 months of age, but mostly do not go on to develop kidney failure. The disease is caused by a defect in the structure of the type-IV collagen fibrils of the glomerular basement membrane. As a consequence, the collagen fibrils of the glomerular basement membrane are unable to form cross-links, so the structural integrity is weakened and the membrane is more susceptible to "wear-and-tear" damage. As the structure of the basement membrane begins to degenerate, plasma proteins are lost in the urine and symptoms begin to appear. Affected males appear healthy for the first three months of life, but then symptoms start to appear and worsen as the disease progresses: the dog becomes lethargic and muscle wastage occurs, as a result of proteinuria. From three months of age onwards, a reduced glomerular filtration rate is detected, indicative of progressive kidney failure.
Clinically, proteinuria is found in both sexes from the age of three to four months; in dogs older than this, kidney failure in combination with more or less pronounced hearing loss occurs swiftly and death at the age of 8 to 15 months is expected. In heterozygous females, the disease develops slowly. The disease can be treated to slow down the development by use of cyclosporine A and ACE inhibitors, but not be stopped.
If a carrier female is mated with a healthy stud dog, the female offspring have a 50% chance of being carriers for the disease, and any male offspring have a 50% chance of being affected by the disease. A genetic test is available for this disease.
Samoyed (dog)