{"id":218449,"date":"2024-09-12T07:23:50","date_gmt":"2024-09-12T05:23:50","guid":{"rendered":"http:\/\/staging.laboklin.com\/labogen\/labogen_wp\/hereditaere-ataxie-ha"},"modified":"2025-07-23T09:06:58","modified_gmt":"2025-07-23T07:06:58","slug":"hereditaere-ataxie-ha","status":"publish","type":"page","link":"https:\/\/labogen.com\/uk\/erbkrankheiten-hund\/hereditaere-ataxie-ha\/","title":{"rendered":"Heredit\u00e4re Ataxie (HA) – Australian Shepherd, Bobtail, Gordon Setter, Mini Aussie, Norwegischer Buhund, Norwegischer Elchhund"},"content":{"rendered":"

Hereditary ataxia (HA)<\/h1>

General description<\/strong><\/h4>

Hereditary ataxia (HA) is a progressive disease, clinical signs are hypermetria, loss of balance, incoordination of gait and intention tremor, which later progress to severe gait disturbances. In Old English Sheepdogs and Gordon Setters, affected dogs will show first indications of cerebellar neurodegeneration at an age between six months and up to four years; in Norwegian Elkhounds and Norwegian Buhund symptoms develop at the age of 4 to 20 weeks, while affected Australian Shepherds and Miniature American Shepherds show symptoms at the age between 4 and 19 months. <\/p>

Breeds<\/span><\/strong><\/h4>

Australian Shepherd, Gordon Setter, Miniature American Shepherd, Norwegian Buhund, Norwegian Elkhound , Old English Sheepdog<\/p>

Detailed description<\/summary>

Hereditary ataxia (HA) is a progressive disease with clinical signs like hypermetria, loss of balance, incoordination of gait and intention tremor, which later progress to severe gait disturbances.<\/p><\/details><\/div><\/div>

<\/div><\/div>

Hereditary Ataxia (HA) - Old English Sheepdog, Gordon Setter<\/h4>

In the breeds Old English Sheepdog and Gordon Setter first symptoms appear within the age of 5 months to 4 years. A variant in the RAB24 gene was identified to cause HA in these breeds. <\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8449<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>7-14 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Age of onset<\/strong><\/td>5 months - 4 years<\/td><\/tr>
Causality<\/strong><\/td>causally<\/td><\/tr>
Gene<\/strong><\/td>RAB24<\/td><\/tr>
Mutation<\/strong><\/td>A-C<\/td><\/tr>
Literature<\/strong><\/td>OMIA:001913-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>

Hereditary Ataxia (HA) - Australian Shepherd<\/h4>

In the breed Australian Shepherd and Miniature American Shepherd, first signs like hypermetria, bunny-hopping and a wobbly and stiff gait on the pelvic limbs can be seen between 4 and 19 months. These uncoordinated movements and spasticity are worsening progressively and leading to inability to walk at the age of 30 to 44 months. Brain histology results revealed diffuse demyelination. A mutation in the PNPLA8 gene was identified to cause HA in this breed.<\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8449<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>7-14 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Age of onset<\/strong><\/td>4-19 months <\/td><\/tr>
Causality<\/strong><\/td>causally<\/td><\/tr>
Gene<\/strong><\/td>PNPLA8<\/td><\/tr>
Mutation<\/strong><\/td>INS<\/td><\/tr>
Literature<\/strong><\/td>OMIA:000827-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>

Hereditary Ataxia (HA) - Norwegian Elkhound <\/h4>

A mutation in the HACE1 gene causes HA in the breed Norwegian Elkhound. Affected puppies show clinical symptoms at an age between 4 and 20 weeks. The puppies slipped easily on the ground, occasionally fell over and had a hanging tail atypical for the breed. <\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8449<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>7-14 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Causality<\/strong><\/td>causally<\/td><\/tr>
Gene<\/strong><\/td>HACE1<\/td><\/tr>
Mutation<\/strong><\/td>DEL<\/td><\/tr>
Literature<\/strong><\/td>OMIA:002522-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>

Hereditary Ataxia (HA) - Norwegian Buhund<\/h4>

A mutation in the KCNIP4 gene causes HA in the breed Norwegian Buhund. Affected puppies show clinical symptoms at an age between 4 and 20 weeks. The puppies slipped easily on the ground, occasionally fell over and had a hanging tail atypical for the breed. <\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8449<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>7-14 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Age of onset<\/strong><\/td>4-20 weeks<\/td><\/tr>
Causality<\/strong><\/td>causally<\/td><\/tr>
Gene<\/strong><\/td>KCNIP4<\/td><\/tr>
Mutation<\/strong><\/td>T-C<\/td><\/tr>
Literature<\/strong><\/td>OMIA:002240-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>
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