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

207780

Sigma-Aldrich

Copper, powder, <75 μm, 99%

326429

Sigma-Aldrich

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61139

Sigma-Aldrich

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326488

Sigma-Aldrich

Copper, beads, 2-8 mm, ≥99.99% trace metals basis

326410

Sigma-Aldrich

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349224

Sigma-Aldrich

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

349216

Sigma-Aldrich

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12816

Sigma-Aldrich

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254177

Sigma-Aldrich

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

266744

Sigma-Aldrich

Copper, foil, thickness 1.0 mm, 99.999% trace metals basis

349151

Sigma-Aldrich

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BCR058

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

349208

Sigma-Aldrich

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

GF23864463

Copper, tube, 1000mm, outside diameter 0.75mm, inside diameter 0.23mm, wall thickness 0.26mm, as drawn, 99.9%

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GF94832208

Copper, tube, 1000mm, outside diameter 1.0mm, inside diameter 0.24mm, wall thickness 0.38mm, as drawn, 99.9%

GF26383349

Copper - O.F.H.C., foil, 5m coil, thickness 0.125mm, hard, 99.95+%

GF53132330

Copper - O.F.H.C., foil, 50mm disks, thickness 0.075mm, half hard, 99.95+%

GF09290426

Copper - O.F.H.C., foil, 300x300mm, thickness, 0.5mm, half hard, flattened, 99.95+%

GF47171033

Copper, wire reel, 100m, diameter 0.025mm, as drawn, 99.99+%

GF24248864

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

GF69818855

Copper - O.F.H.C., foil, 50mm disks, thickness 0.05mm, hard, 99.95+%

GF10253179

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%

GF43853148

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