Cone-Rod Dystrophy (Discovered in the Standard Wirehaired Dachshund; crd SWD)
Cone-Rod Dystrophy (Discovered in the Standard Wirehaired Dachshund; crd SWD). Autosomal recessive. Observed in 0 of 266 breeds tested in the Sniff Atlas, with measured variant frequencies drawn from 242,631 dogs (Donner 2023). Whether a dog carrying this variant is at risk depends on the disease’s inheritance pattern; outcome also depends on penetrance, modifiers, and environment. The frequencies below describe variant prevalence, not confirmed disease incidence.
- OMIA identifier
- OMIA:001455-9615
- InheritanceInheritance patternWhat it isHow the condition is passed down: recessive (two copies needed), dominant (one copy), or more complex.For your dogRecessive means a single-copy carrier is usually healthy but can still pass it on.PreciselyThe documented mode of Mendelian transmission (autosomal recessive or dominant, X-linked, etc.) per OMIA.OMIA · documented
- Autosomal recessive
- Source dataset
- Sniff Atlas v1.0.1 / DOI
A model of human Senior-Loken syndrome 4
This canine condition is studied as a natural model of Senior-Loken syndrome 4 in people; it is not the same as a human Senior-Loken syndrome 4 diagnosis. Studying dogs can move medicine forward for everyone; the canine disease remains the subject.
In people, the disease is described as: Any Senior-Loken syndrome in which the cause of the disease is a mutation in the NPHP4 gene.
In humans it is also called: SLSN4, NPHP4 Senior-Loken syndrome, Senior-Loken syndrome type 4.
Mapped from OMIA via the human disease's OMIM entry to the Mondo Disease Ontology (Monarch Initiative, CC-BY 4.0). Sniff renders this as a model-of link; the canine disease remains the subject of this page.
From OMIA's curated record
Documented in OMIA (Online Mendelian Inheritance in Animals). This describes the disease as recorded in the published literature, not a prediction for any individual dog. As of 2026-08-27.
Summary
Molecular genetics
Human analog
OMIA links this condition to its human counterpart in OMIM (Mendelian Inheritance in Man), the place to read across to the deeper human literature for the same biology.
Source: OMIA (Nicholas, Tammen & the Sydney Informatics Hub), entry OMIA:001455-9615, doi:10.25910/2AMR-PV70 (CC-BY 4.0).
OMIA curates the disease definition, the clinical description and the reference list. The cross-species disease identity is Monarch's. Sniff renders these and adds the breed-level frequencies, the plain-language summary, and a stated reason wherever a number is missing.
How it presents
Catalogued in the Mondo disease ontology (the cross-species disease identity used by the Monarch Initiative) as Senior-Loken syndrome 4 (MONDO:0011756).
Phenotype terms: Human Phenotype Ontology + Mammalian Phenotype Ontology; disease terms: Mondo (Monarch Initiative). Cross-references curated by OMIA (doi:10.25910/2AMR-PV70, CC-BY 4.0).
Published references
The peer-reviewed papers behind this disease, curated by OMIA. Starred entries are OMIA-designated landmark papers. Showing 6 of 9.
- Consensus guidelines for nomenclature of companion animal inherited retinal disorders. · Vet Ophthalmol · 2024 · PMID 38334230
- The Blue Book: Ocular disorders presumed to be inherited in purebred dogs. 13th Edition · https://ofa.org/wp-content/uploads/2022/10/ACVO-Blue-Book-2021.pdf · 2021
- Analysis of a deletion in the nephronophthisis 4 gene in different dog breeds. · Vet Ophthalmol · 2014 · PMID 23998563
- Genetic and phenotypic variations of inherited retinal diseases in dogs: the power of within- and across-breed studies. · Mamm Genome · 2012 · PMID 22065099
- A population study of a mutation allele associated with cone-rod dystrophy in the standard wire-haired dachshund. · Anim Genet · 2009 · PMID 19392817
References curated by OMIA (Nicholas, Tammen & the Sydney Informatics Hub), doi:10.25910/2AMR-PV70 (CC-BY 4.0). Full list at the OMIA entry.
See what Cone-Rod Dystrophy (Discovered in the Standard Wirehaired Dachshund; crd SWD) looks like in your dog's breed.
Observed only in small-sample breeds
Maximum variant frequency per breed across variants in the Donner 2023 cohort, with Wilson 95% confidence intervalsWilson 95% confidence intervalWhat it isThe range the true frequency is probably in. A wide range means we are less sure, usually because few dogs were tested.For your dogTrust tight ranges; treat wide ones as rough estimates.PreciselyA binomial-proportion confidence interval (Wilson score, 95%) that stays reliable at small sample sizes.Sniff Atlas methodology · statistical. The list below is split into well-sampled breeds (n ≥ 50 tested) and small-sample breeds (n < 50, where the Wilson CI typically spans more than 20 percentage points and frequencies should not be compared directly to the well-sampled entries). Frequencies are population-level, not per-litter or per-line.
Scope
This record carries the breed-level carrier frequencies from the Donner 2023 cohort. Penetrance data (the fraction of at-risk dogs that develop the phenotype) is not yet quantified for this disease in the Sniff Atlas v1.0.1. The OMIA entry is the authoritative reference for the clinical phenotype, inheritance pattern, and gene assignment.
Predicted disease relevance at the per-dog level is UNPROVEN. The variant frequency is measured; phenotype outcome depends on penetrance, environment, and modifier loci. Consult a veterinarian for clinical interpretation.
Citations
If you use this record in published work, cite the Sniff Atlas (the published dataset that carries the breed-level carrier frequencies) and the upstream sources:
- Sniff Atlas v1.0.1 for the per-breed carrier frequencies:
Gehring, M. (2026). Sniff Atlas v1.0.1. Zenodo. https://doi.org/10.5281/zenodo.20566358. CC-BY 4.0.
- OMIA for the disease definition, inheritance, and gene assignment:
Nicholas, F. W., & Tammen, I. (2024). OMIA. Sydney Informatics Hub, The University of Sydney. https://doi.org/10.25910/2AMR-PV70. Entry: OMIA:001455-9615.
- Donner et al. 2023 for the breed × variant carrier-frequency cohort:
Donner, J., Freyer, J., Davison, S., Anderson, H., Blades, M., Honkanen, L., et al. (2023). Genetic prevalence and clinical relevance of canine Mendelian disease variants in over one million dogs. PLOS Genetics, 19(2), e1010651. https://doi.org/10.1371/journal.pgen.1010651.
Full citation formats (BibTeX, RIS, CITATION.cff) at sniff.world/cite.
Related
- Sniff Atlas v1.0.1, the source dataset for these frequencies.
- Browse breeds, per-breed Mendelian profiles, including this disease in context.
- OMIA entry OMIA:001455-9615, authoritative clinical reference.
- About OMIA, the catalogue this record comes from, and how Sniff uses it.