The ubiquitously expressed MURR1 protein is absent in canine copper toxicosis

AEM Klomp, B van de Sluis, LWJ Klomp… - Journal of hepatology, 2003 - Elsevier
Background/Aims: Copper toxicosis (CT) in Bedlington terriers is an autosomal recessive
disorder characterized by massive lysosomal copper accumulation in livers of affected dogs,
and a defect in the biliary excretion of this metal. We propose that MURR1, the gene
defective in canine CT, has a role in the regulation of copper excretion into bile during
copper overload. Methods: Polyclonal antibodies raised against full-length recombinant
human MURR1 were used for immunoblot analysis and indirect immunofluorescence …