Functional understanding of the versatile protein copper metabolism MURR1 domain 1 (COMMD1) in copper homeostasis.

Functional understanding of the versatile protein copper metabolism MURR1 domain 1 (COMMD1) in copper homeostasis.

Annals of the New York Academy of Sciences (2014-04-05)

Alina Fedoseienko, Paulina Bartuzi, Bart van de Sluis

ABSTRACT

Copper is an important cofactor in numerous biological processes in all living organisms. However, excessive copper can be extremely toxic, so it is vital that the copper level within a cell is tightly regulated. The damaging effect of copper is seen in several hereditary forms of copper toxicity in humans and animals. At present, Wilson's disease is the best-described and best-studied copper-storage disorder in humans; it is caused by mutations in the ATP7B gene. In dogs, a mutation in the COMMD1 gene has been found to be associated with copper toxicosis. Using a liver-specific Commd1 knockout mouse, the biological role of Commd1 in copper homeostasis has been confirmed. Yet, the exact mechanism by which COMMD1 regulates copper homeostasis is still unknown. Here, we give an overview of the current knowledge and perspectives on the molecular function of COMMD1 in copper homeostasis.

MATERIALS

Product Number

Brand

Product Description

12816

Sigma-Aldrich

Copper, foil, ≥99.8% (complexometric)

BCR058

Continuous cast copper (O), BCR^®, certified reference material, rod

61139

Sigma-Aldrich

Copper, turnings, purum p.a., ≥99.0%

207780

Sigma-Aldrich

Copper, powder, <75 μm, 99%

254177

Sigma-Aldrich

Copper, beads, 2-8 mm, 99.9995% trace metals basis

349216

Sigma-Aldrich

Copper, wire, diam. 1.0 mm, ≥99.9%

349208

Sigma-Aldrich

Copper, foil, thickness 0.025 mm, 99.98% trace metals basis

349224

Sigma-Aldrich

Copper, wire, diam. 2.0 mm, 99.999% trace metals basis

326410

Sigma-Aldrich

Copper, wire, diam. 0.64 mm, 99.995% trace metals basis

349151

Sigma-Aldrich

Copper, foil, thickness 0.5 mm, 99.98% trace metals basis

GF89142701

Copper, tube, 1000mm, outside diameter 5.0mm, inside diameter 4mm, wall thickness 0.5mm, as drawn, 99.9%

GF93436926

Copper, tube, 1000mm, outside diameter 0.95mm, inside diameter 0.29mm, wall thickness 0.33mm, as drawn, 99.9%

GF98886920

Copper, rod, 50mm, diameter 12.7mm, as drawn, 99.99+%

GF87182657

Copper, tube, 1000mm, outside diameter 6.0mm, inside diameter 4mm, wall thickness 1.0mm, as drawn, 99.9%

GF60058346

Copper, sphere, 1000pcs, diameter 3.0mm, precision sphere grade 200

GF69447280

Copper, tube, 100mm, outside diameter 0.75mm, inside diameter 0.23mm, wall thickness 0.26mm, as drawn, 99.9%

GF80997889

Copper - O.F.H.C., foil, 1m coil, thickness 0.010mm, coil width 25mm, hard, 99.95+%

GF58516285

Copper - O.F.H.C., foil, 20m coil, thickness 0.05mm, coil width 10mm, annealed, 99.95+%

GF24248864

Copper - O.F.H.C., foil, 2m coil, thickness 0.20mm, hard, 99.95+%

GF93757786

Copper - O.F.H.C., foil, 15mm disks, thickness 0.125mm, hard, 99.95+%

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Copper - O.F.H.C., foil, 25mm disks, thickness 0.7mm, half hard, 99.95+%

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Copper, wire reel, 0.5m, diameter 0.5mm, as drawn, 99.999%

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Copper, tube, 500mm, outside diameter 2.5mm, inside diameter 1.9mm, wall thickness 0.3mm, as drawn, 99.9%

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Copper, wire reel, 0.2m, diameter 1.0mm, as drawn, 99.99+%

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Copper - O.F.H.C., foil, 2m coil, thickness 0.7mm, half hard, 99.95+%

GF32166761

Copper - O.F.H.C., foil, 5m coil, thickness 0.1mm, coil width 100mm, hard, 99.95+%

GF51978437

Copper, wire reel, 10m, diameter 0.5mm, annealed, 99.9%