The Menkes and Wilson disease genes counteract in copper toxicosis in Labrador retrievers: a new canine model for copper-metabolism disorders
نویسندگان
چکیده
منابع مشابه
Copper-associated chronic hepatitis in Labrador Retrievers.
This study summarizes the clinical and pathologic findings in 15 Labrador Retrievers with copper-associated chronic hepatitis (CACH). Our hypothesis was that this form of hepatitis is caused by a defect in hepatic copper metabolism, which most likely originates from a genetic defect. Affected Labradors consisted of 11 female and 4 male Labrador Retrievers. Eight family members of 2 of these pat...
متن کاملDmm020263 25..38
The deleterious effects of a disrupted copper metabolism are illustrated by hereditary diseases caused by mutations in the genes coding for the copper transporters ATP7A and ATP7B. Menkes disease, involving ATP7A, is a fatal neurodegenerative disorder of copper deficiency. Mutations in ATP7B lead to Wilson disease, which is characterized by a predominantly hepatic copper accumulation. The low i...
متن کاملNew canine models of copper toxicosis: diagnosis, treatment, and genetics.
The One Health principle recognizes that human health, animal health, and environmental health are inextricably linked. An excellent example is the study of naturally occurring copper toxicosis in dogs to help understand human disorders of copper metabolism. Besides the Bedlington terrier, where copper toxicosis is caused by a mutation in the COMMD1 gene, more complex hereditary forms of copper...
متن کاملXIAP Is a copper binding protein deregulated in Wilson's disease and other copper toxicosis disorders.
X-linked inhibitor of apoptosis (XIAP), known primarily for its caspase inhibitory properties, has recently been shown to interact with and regulate the levels of COMMD1, a protein associated with a form of canine copper toxicosis. Here, we describe a role for XIAP in copper metabolism. We find that XIAP levels are greatly reduced by intracellular copper accumulation in Wilson's disease and oth...
متن کاملDietary Management of Labrador Retrievers with Subclinical Hepatic Copper Accumulation
BACKGROUND Genetic and environmental factors, including dietary copper intake, contribute to the pathogenesis of copper-associated hepatitis in Labrador retrievers. Clinical disease is preceded by a subclinical phase in which copper accumulates in the liver. OBJECTIVE To investigate the effect of a low-copper, high-zinc diet on hepatic copper concentration in Labrador retrievers with increase...
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ژورنال
عنوان ژورنال: Disease Models & Mechanisms
سال: 2016
ISSN: 1754-8411,1754-8403
DOI: 10.1242/dmm.020263