Basenji — spontaneous Fanconi syndrome, idiopathic Fanconi syndrome (hereditary; OMIA-verified breed predisposition)

--- license: permission_granted topic_id: companion_breed_health_basenji_spontaneous_fanconi_syndrome_idiopathic_fanconi_syndrome_dog category: companion-breed-health title: "Basenji — spontaneous Fanconi syndrome, idiopathic Fanconi syndrome (hereditary; OMIA-verified breed predisposition)" lang: en source: "OMIA (Online Mendelian Inheritance in Animals, University of Sydney) breed-specific hereditary-disorder record, saved verbatim to source_file; C1 substring-verified. Pulled 2026-08-02 from local OMIA database dump." source_file: pdf-raw/breed-health/basenji_spontaneous_fanconi_syndrome_idiopathic_fanconi_syndrome_5309.txt date_parsed: 2026-08-02 tokens_estimated: 382 verification: method: substring_match claims: 7 passed: 7 date: 2026-08-02 recovered: false path: companion-breed-health/companion_breed_health_basenji_spontaneous_fanconi_syndrome_idiopathic_fanconi_syndrome_dog/01_companion_breed_health_basenji_spontaneous_fanconi_syndrome_idiopathic_fanconi_syndrome_dog.md source_document: "OMIA — Online Mendelian Inheritance in Animals (University of Sydney)" citation: authority: "OMIA — Online Mendelian Inheritance in Animals (University of Sydney)" title: "Basenji — spontaneous Fanconi syndrome, idiopathic Fanconi syndrome (hereditary; OMIA-verified breed predisposition)" url: "https://omia.org/OMIA002683/9615/" retrieved: "2026-08-02" ref: "OMIA breed-specific hereditary-disorder records (omia.org), derived from local OMIA database dump; dataset: https://doi.org/10.25910/2AMR-PV70" doc_type: "academic animal-genetics database (breed-specific disorder entries)" needs_review: false

Basenji — spontaneous Fanconi syndrome, idiopathic Fanconi syndrome (hereditary; OMIA-verified breed predisposition)

Source: first-hand OMIA (University of Sydney) breed-specific hereditary-disorder record, saved verbatim to source_file; every claim below is a C1 byte-substring of it.

Claims

• Breed: Basenji (Dog)
• Disorder: spontaneous Fanconi syndrome, idiopathic Fanconi syndrome
• Mode of inheritance: Autosomal recessive
• Summary: Information listed here was previously listed under OMIA:000366-9615 : Fanconi syndrome in Canis lupus familiaris
• Clin feat: Fanconi syndrome is a rare condition in dogs which results in abnormal renal function. More specifically, it causes reduced function of the proximal convoluted tubules (Yearley et al., 2004), resulting in reduced reabsorption of water, nutrients and electrolytes from the tubular fluid and their eventual loss in urine. The clinical consequences of this disease for the animal include polyuria and polydipsia, dehydration, weight loss and muscle weakness (Yearley et al., 2004). Most cases progress to renal failure within a few years of the onset of initial clinical signs Bovée et al. (1978, PMID:684432). IT thanks DVM student Harry Cahill, who provided the basis of this contribution in May 2023.
• Defect: yes
• Gen test: The a href=https://ofa.org/diseases/?breed=BJwebsite of the Orthopedic Foundation for Animals (OFA)/a reports the availability of a DNA test for this disorder in the Basenji breed. The basis of this test was initially described in a PhD thesis by Farias (2011) and a conference proceeding (Farias et al., 2012). Thanks to Mario Van Poucke and Kathy Davies for alerting FN to this thesis. The detailed information was published in 2024 (Farias et al. 2024).

Associated gene(s)

Derived from OMIA database dump (omia.xml, local); structured field, not verbatim prose. First-hand values below are verbatim substrings of the topic's pdf-raw/omia/<phene_id>.txt source file.

• Gene: MTMR15 (Entrez Gene ID 388246875) — OMIA Phene_Gene / GeneSynonym
• OMIA molecular-genetics note: In an unpublished thesis, Farias (2011) reported whole-genome sequencing one affected Basenji and three unaffected dogs from other breeds, which eventually led to identification of a candidate causal mutation: "317 bp of exon 14 were deleted starting at the second exon14 nucleotide and extending into the 3’ untranslated region of FAN1" (the gene encoding Fanconi anemia-associated nuclease 1). This…

Evidence (references)

Derived from OMIA database dump (omia.xml, local); structured field, not verbatim prose. First-hand values below are verbatim substrings of the topic's pdf-raw/omia/<phene_id>.txt source file.

• 1978. The Fanconi syndrome in Basenji dogs: a new model for renal transport defects. Science — PubMed:PMID684432 | DOI:10.1126/science.684432 — OMIA Phene_Article / Article
• 1976. Glucosuria associated with renal tubular dysfunction in three Basenji dogs. J Am Vet Med Assoc — PubMed:PMID1270337 — OMIA Phene_Article / Article
• 1990. Prevalence and geographic distribution of Fanconi syndrome in Basenjis in the United-States. J Am Vet Med Assoc — PubMed:PMID2391269 — OMIA Phene_Article / Article
• 1992. Membrane fluidity and sodium transport by renal membranes from dogs with spontaneous idiopathic Fanconi syndrome. Metabolism — PubMed:PMID1542263 | DOI:10.1016/0026-0495(92)90267-e — OMIA Phene_Article / Article
• 1994. Renal brush border membrane lipid composition in Basenji dogs with spontaneous idiopathic Fanconi syndrome. Metabolism — PubMed:PMID8084281 | DOI:10.1016/0026-0495(94)90047-7 — OMIA Phene_Article / Article
• 2005. [Inventory of Fanconi syndrome in Basenji dogs in The Netherlands]. Tijdschr Diergeneeskd — PubMed:PMID16130757 — OMIA Phene_Article / Article
• 2004. Survival time, lifespan, and quality of life in dogs with idiopathic Fanconi syndrome. J Am Vet Med Assoc — PubMed:PMID15328712 | DOI:10.2460/javma.2004.225.377 — OMIA Phene_Article / Article
• 1989. Cystinuria in dogs: comparison of the cystinuric component of the Fanconi syndrome in Basenji dogs to isolated cystinuria. Metabolism — PubMed:PMID2909832 | DOI:10.1016/0026-0495(89)90173-x — OMIA Phene_Article / Article
• 1982. Renal tubular defects of spontaneous Fanconi syndrome in dogs. Prog Clin Biol Res — PubMed:PMID7122624 — OMIA Phene_Article / Article
• 1981. Proline and glucose transport by renal membranes from dogs with spontaneous idiopathic Fanconi syndrome. Proc Natl Acad Sci U S A — PubMed:PMID6950417 | DOI:10.1073/pnas.78.12.7769 — OMIA Phene_Article / Article
• 1979. Characterization of renal defects in dogs with a syndrome similar to the Fanconi syndrome in man. J Am Vet Med Assoc — PubMed:PMID438041 — OMIA Phene_Article / Article
• 1978. Spontaneous Fanconi syndrome in the dog. Metabolism — PubMed:PMID619225 | DOI:10.1016/0026-0495(78)90122-1 — OMIA Phene_Article / Article
• (4 additional references in OMIA)

Comparative medicine (human OMIM)

Derived from OMIA database dump (omia.xml, local); structured field, not verbatim prose. First-hand values below are verbatim substrings of the topic's pdf-raw/omia/<phene_id>.txt source file.

• OMIM:613534 (type: gene) — OMIA Group_OMIM (via OMIA_ID)
• OMIM:614817 (type: trait) — OMIA Group_OMIM (via OMIA_ID)

verification_derived: method: derived_from_dataset source: "OMIA database dump (omia.xml, local); fields Gene/Variant/Article/OMIM" note: "Structured fields (gene, variant rsID/protein change, PubMed/DOI references, OMIM cross-link) extracted from OMIA dump and presented with first-hand values; not verbatim prose."