Reagent | Copper
A Unique Test for the Determination of Copper
Benefits of the Randox Copper Assay
A correlation coefficient of r=0.97 was displayed when the Randox copper assay was compared to commercially available methods.
The Randox copper assay displayed a precision of <2.15% CV.
Wide measuring range
The Randox copper assay has a measuring range of 6.6 – 86µmol/l for the comfortable detection of clinically important results.
Standard supplied with the kit
The Randox copper kit includes the standard simplifying the ordering process. Calibrator is available for automated use.
Controls available offering a complete testing package.
Applications available detailing instrument-specific settings for the convenient use of the Randox copper assay on a variety of clinical chemistry analysers.
Copper (CU) is an essential trace mineral, naturally available in some foods and as dietary supplements. CU is a cofactor for several enzymes, known as cuproenzymes, which are involved in connective tissue synthesis, energy production, iron metabolism, neuropeptide activation and neurotransmitter synthesis. CU is also involved in brain development, immune system functioning, neurohormone homeostasis, pigmentation, regulation of gene expression, and several physiological processes, such as angiogenesis 1.
CU has been recognised as both an antioxidant and pro-oxidant. Naturally occurring within the body, free radicals interact with genetic material, damage cell walls and contribute to the development of several health problems. As an antioxidant, CU scavenges to neutralise the free radicals, aiding in the prevention of oxidative damage. Conversely, as a pro-oxidant, CU can promote free radical damage, inducing the development of health problems such as Alzheimer’s disease. Consequently, CU is vital as part of a balanced diet 2.
CU deficiency in Western countries is rare, however, altered CU metabolism may influence CU deficiency which negatively impacts the connective tissue, nervous, immune and cardiovascular systems. Such conditions that can predispose CU deficiency include: prematurity, gastric bypass, burns, over-the-counter vitamins containing zinc and iron and infants fed with unmodified cow milk 3.
Menkes disease is a rare x-linked recessive disorder of CU metabolism caused by mutations to the ATP7A gene. Menkes disease affects an estimated 1 in every 100,000 – 250,000 births and is characterised by sparse, kinky hair and failure to thrive and progressive deterioration of the nervous system. Symptoms commonly present during infancy, but, in some cases, the symptoms may present in early to middle childhood. If treatment is started early, the prognosis may improve 4.
Copper toxicity is also rare but can be caused by consuming too many dietary supplements high in copper, drinking contaminated water and from fungicides containing CU sulphates 3.
Wilson’s disease is an autosomal recessive disorder caused by mutations to the ATP7B gene, which is highly expressed in the liver, kidneys and placenta. Wilson’s disease affects approximately 1 in every 40,000 and is characterised by hepatic, neuropsychiatric and ophthalmic symptoms as a result of excess copper accumulation. Unlike most genetic diseases, early detection and implementation of a treatment plan for those with Wilson’s disease can prevent longer term morbidity due to copper induced end organ dysfunction 3.
Clinical Chemistry Calibrator
Chemistry Premium Controls
Clinical Chemistry EQA
 Rare Diseases. Menkes disease. https://rarediseases.info.nih.gov/diseases/1521/menkes-disease (accessed 10 March 2020).
This year, Randox Reagents are supporting Rare Disease Day on 28th February. Randox offer a test that aids in the diagnosis and monitoring of Wilson Disease and Menkes Disease which are rare inherited disorders of copper metabolism.
What is a rare disease?
According to the European Union, a rare disease is defined as a disease that affects less than 5 in 10,000 of the general population. 7% of the population will be affected by a rare disease at some point in their life. This equates to 30 million people in Europe.
Wilson Disease is a rare inherited autosomal recessive disorder of copper metabolism, characterised by excessive deposition of copper in various bodily tissues, particularly the liver, brain, and corneas of the eyes. This is due to mutations of the ATP7B gene which is responsible for encoding specific proteins that are responsible for the transportation of copper from the liver around the body, which is prohibited due to the mutations. If left untreated, Wilson Disease can cause hepatic disease, central nervous system dysfunction, or death. Approximately 1 in 30,000 people are affected by Wilson Disease worldwide (WDA, 2018). The first sign of Wilson Disease is liver dysfunction in more than half of patients, beginning at six years of age, however, it usually presents clinically in teenage years or early twenties manifesting as acute hepatitis. Some individuals with Wilson Disease have been thought to have infectious hepatitis or infectious mononucleosis and so it is vital that those with unexplained, abnormal liver tests are tested for Wilson Disease.
Menkes Disease is more likely to affect premature babies and is a rare inherited x-link recessive disorder of copper metabolism, characterised by sparse, kinky hair; failure to gain weight and grow at the expected rate (failure to thrive); and deterioration of the nervous system. This is due to mutations of the ATP7A gene which is responsible for the absorption of copper from food in the small intestines and supplying copper to certain enzymes that are critical for the structure of bone, skin, hair, blood vessels, and the nervous system. Approximately 1 in 100,000 people are affected by Menkes disease worldwide (USA National Library of Medicine, 2018). The first sign of Menkes Disease develops at 2-3 months of age and includes curly, sparse, coarse, dull, and discoloured haired.
As there are no cures for Wilson Disease or Menkes Disease, treatment aids to reduce/replace copper within the body. The Randox Copper assay can comfortably detect copper levels outside of the healthy range to aid in the diagnosis and monitoring of treatment of Wilson Disease and Menkes Disease.
Randox Copper Assay
The Randox Copper assay is used to measure the amount of copper in the blood; to help with the diagnosis and monitoring of rare inherited diseases related to copper toxicity (Wilson Disease) and copper deficiency (Menkes Disease). Copper deficiency is less likely because a normal diet contains plenty of copper including organ meats, beans, and wholegrains, however, copper deficiency is more likely to occur in those who are malnourished, more likely children.
For more information visit: https://www.randox.com/copper
To request an application for your specific analyser, contact firstname.lastname@example.org