A Frameshift Mutation in Golden Retriever Dogs with Progressive Retinal Atrophy Endorses SLC4A3 as a Candidate Gene for Human Retinal Degenerations
2011

Mutation in SLC4A3 Linked to Retinal Degeneration in Golden Retrievers

Sample size: 945 publication 10 minutes Evidence: high

Author Information

Author(s): Downs Louise M., Wallin-Håkansson Berit, Boursnell Mike, Marklund Stefan, Hedhammar Åke, Truvé Katarina, Hübinette Louise, Lindblad-Toh Kerstin, Bergström Tomas, Mellersh Cathryn S.

Primary Institution: Canine Genetics, Animal Health Trust, Newmarket, United Kingdom

Hypothesis

Is a frameshift mutation in the SLC4A3 gene responsible for progressive retinal atrophy in Golden Retrievers?

Conclusion

The study identifies a frameshift mutation in the SLC4A3 gene as a significant cause of progressive retinal atrophy in Golden Retrievers.

Supporting Evidence

  • The mutation was found in 56% of PRA cases and 87% of obligate carriers.
  • Genome-wide association analysis revealed a significant association on chromosome 37.
  • Allele frequencies for the mutation were approximately 4% in the UK and 6% in Sweden.

Takeaway

Some Golden Retrievers go blind because of a gene mutation that affects their eyes, similar to a condition that can happen in people.

Methodology

Genome-wide association study with 27 PRA cases and 19 controls, followed by mutation screening in 945 Golden Retrievers.

Limitations

A large proportion of PRA cases remain unexplained, indicating genetic heterogeneity.

Participant Demographics

Golden Retrievers from the UK, Sweden, France, and the USA.

Statistical Information

P-Value

p=6.9×10−7

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1371/journal.pone.0021452

Want to read the original?

Access the complete publication on the publisher's website

View Original Publication