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Inherited disorders similar to human <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content> have been reported in different mammalian species. In the present study, we investigated a female mixed\u2010breed dog with clinical signs of <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content>. Whole\u2010genome sequencing of the affected dog revealed two missense variants in the <jats:italic><jats:styled-content style=\"fixed-case\">TNXB<\/jats:styled-content><\/jats:italic> gene, encoding the extracellular matrix protein tenascin <jats:styled-content style=\"fixed-case\">XB<\/jats:styled-content>. In humans, <jats:italic><jats:styled-content style=\"fixed-case\">TNXB<\/jats:styled-content><\/jats:italic> genetic variants cause classical\u2010like <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content> or the milder hypermobile <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content>. The affected dog was heterozygous at both identified variants. Each variant allele was transmitted from one of the case's parents, consistent with compound heterozygosity. Although one of the variant alleles, <jats:styled-content style=\"fixed-case\">XM<\/jats:styled-content>_003431680.3:c.2012G&gt;A, p.(Ser671Asn), was private to the family of the affected dog and absent from whole\u2010genome sequencing data of 599 control dogs, the second variant allele, <jats:styled-content style=\"fixed-case\">XM<\/jats:styled-content>_003431680.3:c.2900G&gt;A, p.(Gly967Asp), is present at a low frequency in the Chihuahua and Poodle population. Given that <jats:italic><jats:styled-content style=\"fixed-case\">TNXB<\/jats:styled-content><\/jats:italic> is a functional candidate gene for <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content>, we suggest that compound heterozygosity for the identified <jats:italic><jats:styled-content style=\"fixed-case\">TNXB<\/jats:styled-content><\/jats:italic> variants may have caused the <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content>\u2010like phenotype in the affected dog. Chihuahuas and Poodles should be monitored for <jats:styled-content style=\"fixed-case\">EDS<\/jats:styled-content> cases, which might confirm the hypothesized pathogenic effect of the segregating <jats:italic><jats:styled-content style=\"fixed-case\">TNXB<\/jats:styled-content><\/jats:italic> variant.<\/jats:p>","DOI":"10.1111\/age.12830","type":"journal-article","created":{"date-parts":[[2019,7,31]],"date-time":"2019-07-31T13:20:31Z","timestamp":1564579231000},"page":"546-549","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Compound heterozygosity for <i><scp>TNXB<\/scp><\/i> genetic variants in a mixed\u2010breed dog with Ehlers\u2010Danlos syndrome"],"prefix":"10.1111","volume":"50","author":[{"given":"A.","family":"Bauer","sequence":"first","affiliation":[{"name":"Vetsuisse Faculty Institute of Genetics University of Bern 3001 Bern Switzerland"},{"name":"DermFocus University of Bern 3001 Bern 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