Kupfertoxikose (CT) - Labrador Retriever
SKU: 3162844118

Kupfertoxikose (CT) - Labrador Retriever

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Kupfertoxikose (CT) - Labrador RetrieverStoffwechselstrungen des Kupfers sind genetische Strungen des Kupferstoffwechsels in der Leber, die durch Mutationen in zwei Genen verursacht werden, die Kupfertransporter kodieren ATP7A und ATP7B. Der ATP7A Transporter ist entscheidend fr den Kupfertransport von Fibroblasten in die Leber und der ATP7B ist entscheidend fr den Kupferexport aus der Leber. Mutationen im ATP7A fhren zu einem defekten ATP7A Transporter, sodass das Kupfer nicht richtig aus

Stoffwechselstörungen des Kupfers sind genetische Störungen des Kupferstoffwechsels in der Leber, die durch Mutationen in zwei Genen verursacht werden, die Kupfertransporter kodieren – ATP7A und ATP7B. Der ATP7A-Transporter ist entscheidend für den Kupfertransport von Fibroblasten in die Leber und der ATP7B ist entscheidend für den Kupferexport aus der Leber. Mutationen im ATP7A führen zu einem defekten ATP7A-Transporter, sodass das Kupfer nicht richtig aus den Fibroblasten exportiert und in die Leber importiert werden kann, was zu einem Kupfermangel in der Leber führt. Dies führt zu Menkes-Krankheit, einer tödlichen neurodegenerativen Erkrankung, die durch zerebrale und zerebelläre Degeneration, Gedeihstörung, grobes Haar und Bindegewebsanomalien gekennzeichnet ist. Währenddessen führen die Mutationen im ATP7B-Gen zu einem defekten ATP7B-Transporter, sodass das Kupfer nicht richtig aus der Leber exportiert werden kann, was zu einer Kupferansammlung in der Leber und sekundär im Gehirn führt. Dies führt zu Morbus Wilson, was bei mittelalten Hunden zu Zirrhose und neuronaler Degeneration führt. Die Mutation im ATP7A-Gen hat eine X-chromosomale Vererbung und die Mutation im ATP7B-Gen hat eine unvollständig dominante Vererbung. Störungen treten bei Labrador Retrievern und Dobermann Pinschern auf. Bei Dobermann Pinschern werden jedoch Mutationen in beiden Genen gefunden, aber nur die Mutation im ATP7B kann zu einer Störung führen, in diesem Fall Morbus Wilson. Bei Labrador Retrievern können Mutationen nur in einem oder in beiden Genen gleichzeitig vorhanden sein. Mutationen in beiden Genen gleichzeitig können zu einer abnorm hohen Kupferansammlung führen, die zu Kupfertoxikose führt, insbesondere bei Hündinnen.

Vererbung: komplex

Mutation: ATP7A und ATP7B Gen

Gentest: Die Methode, die für den Gentest verwendet wird, ist äußerst genau und ermöglicht eine vollständige Unterscheidung zwischen betroffenen Tieren, Trägern und gesunden Hunden. DNA-Tests können in jedem Alter durchgeführt werden.

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