Skip to content
Back

Fanconi Syndrome

Fanconi Syndrome (FAN) is an inherited disorder in dogs that affects kidney function, leading to abnormal reabsorption of nutrients and electrolytes.

Affected Genes: FAN1

Inheritance: Autosomal Recessive

Variant(canFam6):
chr3:40698091-40698407: 317 bp deletion

Breed: Basenji

General Information: Fanconi Syndrome (FAN) is a genetic kidney disorder that impairs the kidney’s ability to reabsorb essential nutrients and electrolytes from the urine back into the bloodstream. Affected dogs typically present with symptoms between four and eight years of age. These symptoms include increased urination, excessive drinking, weight loss despite a normal appetite, weakness, urinary incontinence, and poor hair coat. A hallmark of the disease is the presence of glucose in the urine despite normal blood glucose levels. If untreated, Fanconi Syndrome can progress to chronic kidney failure and significantly impact the dog's overall health. Treatment can manage the condition and improve the quality of life, but it cannot cure the disease. The median survival time after diagnosis is around five years, with most affected dogs living up to 12 years.

How to Read Your Dog's Test Results for this Genetic Variant:

Two Variants Detected: Dog Likely Affected

One Variant Detected: Dog Unlikely Affected

No Variants Detected: No Effect

Gene / Testing Information: Genetic testing for Fanconi Syndrome (FAN) involves screening for mutations in the FAN1 gene to determine carrier status. This disorder is inherited in an autosomal recessive manner, meaning a dog must inherit two copies of the mutated gene, one from each parent, to develop the disease. Carriers do not typically show symptoms but can pass the mutation to their offspring. When two carriers are bred, each puppy has a 25% chance of being affected and a 50% chance of being a carrier. To prevent producing affected puppies and eliminate the mutation from breeding lines, it is crucial to avoid breeding two carriers. Genetic testing is essential for responsible breeding practices, ensuring that dogs not carrying the mutation do not pose a risk of producing affected puppies.

References:
Johnson GS, Farias FH, Mhlanga-Mutangadura T, Taylor JF, O'Brien DP, Schnabel RD. Whole genome sequencing reveals a deletion of the last exon of FAN1 in Basenji dogs with adult-onset Fanconi syndrome [abstract]. Presented at the 63rd Annual Meeting of the American Society of Human Genetics in San Francisco, California; 2012 Nov 6-10.

Noonan CH, Kay JM. Prevalence and geographic distribution of Fanconi syndrome in Basenjis in the United States. J Am Vet Med Assoc. 1990 97(3):345-349.

Yearley JH, Hancock DD, Mealey KL. Survival time, lifespan, and quality of life in dogs with idiopathic Fanconi syndrome. J Am Vet Med Assoc. 2004 225(3):377-383.