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)

207780

Sigma-Aldrich

Copper, powder, <75 μm, 99%

349216

Sigma-Aldrich

Copper, wire, diam. 1.0 mm, ≥99.9%

326429

Sigma-Aldrich

Copper, wire, diam. 0.25 mm, 99.999% trace metals basis

254177

Sigma-Aldrich

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

349151

Sigma-Aldrich

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

349224

Sigma-Aldrich

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

266744

Sigma-Aldrich

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

326410

Sigma-Aldrich

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

BCR058

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

326488

Sigma-Aldrich

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

61139

Sigma-Aldrich

Copper, turnings, purum p.a., ≥99.0%

GF29197480

Copper - O.F.H.C., tube, 1000mm, outside diameter 10mm, inside diameter 8mm, wall thickness 1.0mm, 99.95+%

GF59430304

Copper, mesh, 150x150mm, nominal aperture 0.14mm, thickness 0.25mm, wire diameter 0.115mm, 100x100 wires/inch, open area 30.3%, plain weave mesh

GF53474980

Copper, tube, 100mm, outside diameter 12.7mm, inside diameter 10.26mm, wall thickness 1.22mm, as drawn, 99.9%

GF07134960

Copper, insulated wire, 50m, conductor diameter 0.125mm, insulation thickness 0.01mm, polyimide insulation, 99.99%

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GF29608904

Copper, insulated wire, 50m, conductor diameter 0.45mm, insulation thickness 0.25mm, PTFE (polytetrafluoroethylene) insulation

GF06390525

Copper - O.F.H.C., tube, 200mm, outside diameter 0.38mm, inside diameter 0.22mm, wall thickness 0.08mm, 99.95+%

GF01913419

Copper - O.F.H.C., foil, 0.5m coil, thickness 0.006mm, hard, 99.95+%

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Copper, insulated wire, 2m, conductor diameter 1.0mm, insulation thickness 0.25mm, PTFE (polytetrafluoroethylene) insulation

GF92764974

Copper - O.F.H.C., foil, 50x50mm, thickness 0.1mm, hard, 99.95+%

GF56099571

Copper, insulated wire, 200m, conductor diameter 0.5mm, insulation thickness 0.06mm, polyimide insulation