{"id":218007,"date":"2024-09-12T07:23:47","date_gmt":"2024-09-12T05:23:47","guid":{"rendered":"http:\/\/staging.laboklin.com\/labogen\/labogen_wp\/globoidzellleukodystrophie"},"modified":"2024-09-12T07:23:47","modified_gmt":"2024-09-12T05:23:47","slug":"globoidzellleukodystrophie","status":"publish","type":"page","link":"https:\/\/labogen.com\/fi\/erbkrankheiten-hund\/globoidzellleukodystrophie\/","title":{"rendered":"Globoidzellleukodystrophie"},"content":{"rendered":"

Globoid cell leukodystrophy (Krabbe-disease)<\/h1>

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

Symptoms of globoidcell leucodystrophy are ataxia, paresis of the hind legs and neurologic degeneration. After onset (1-3months) the disease progresses. Affected animals are usually euthanased till to the age of 10 months.<\/p>

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

Cairn Terrier, Irish Red Setter, West Highland White Terrier<\/p>

Detailed description<\/summary>

Globoid cell leucodystrophy is a lipid storage disorder with progressive degeneration of the white substance of the central nervous system<\/strong> (CNS). The disease is caused by a genetically caused lack of the enzyme galactocerebroside betagalactosidase (GALC). This enzyme is responsible for the lysosomal metabolism of certain galactolipids. Due to the lack of enzyme, these lipids are deposited in the CNS particularly in the multi-nucleated giant cells (globoid cells).<\/p>

The globoid cell leucodystrophy manifests itself in affected dogs at the age of 1-3 months<\/strong>, beginning with ataxia and paresis of the hind legs<\/strong>. During the progress of the disease, muscular atrophy<\/strong> and neurological degeneration<\/strong> oocur. Due to the lack treatment possibilities, the affected animals are usually euthanized after 10 months at the latest. <\/p><\/p><\/details><\/div><\/div>

<\/div><\/div>

Globoid Cell Leukodystrophy - Irish Red Setter<\/h4>

The symptoms described in this breed can be found in the text above.<\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8007<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>3-5 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>1-3 months<\/td><\/tr>
Causality<\/strong><\/td>causally<\/td><\/tr>
Gene<\/strong><\/td>GALC<\/td><\/tr>
Mutation<\/strong><\/td>T-G<\/td><\/tr>
Literature<\/strong><\/td>OMIA:000578-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>

Globoid Cell Leukodystrophy - Terrier<\/h4>

The symptoms described in this breed can be found in the text above.<\/p><\/div>

Order details<\/summary>
Test number<\/strong><\/span><\/td>8007<\/td><\/tr>
Sample material<\/strong><\/td>0.5 ml EDTA blood, 2x cheek swab, 1x special swab (eNAT)<\/td><\/tr>
Test duration<\/strong><\/td>3-5 working days<\/td><\/tr><\/table><\/details>
Test specifications<\/summary>
Symptom complex<\/strong><\/td>neurological<\/td><\/tr>
Inheritance<\/strong><\/td>autosomal recessive<\/td><\/tr>
Gene<\/strong><\/td>GALC<\/td><\/tr>
Mutation<\/strong><\/td>T-G<\/td><\/tr>
Literature<\/strong><\/td>OMIA:000578-9615<\/a><\/td><\/tr><\/table><\/details><\/div>
Order now ...<\/a><\/strong><\/div><\/div><\/div>
<\/div><\/div>
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