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

RÉSUMÉ

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.

MATÉRIAUX

Référence du produit

Marque

Description du produit

203122

Sigma-Aldrich

Copper, powder, 99.999% trace metals basis

349178

Sigma-Aldrich

Copper, foil, thickness 0.25 mm, 99.98% trace metals basis

266086

Sigma-Aldrich

Copper, powder, <425 μm, 99.5% trace metals basis

349216

Sigma-Aldrich

Copper, wire, diam. 1.0 mm, ≥99.9%

207780

Sigma-Aldrich

Copper, powder, <75 μm, 99%

774111

Sigma-Aldrich

Copper, nanopowder, 40-60 nm particle size (SAXS), ≥99.5% trace metals basis

326453

Sigma-Aldrich

Copper, powder (spheroidal), 10-25 μm, 98%

326429

Sigma-Aldrich

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

774103

Sigma-Aldrich

Copper, nanopowder, 60-80 nm particle size (SAXS), ≥99.5% trace metals basis

349208

Sigma-Aldrich

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

61139

Sigma-Aldrich

Copper, turnings, purum p.a., ≥99.0%

12816

Sigma-Aldrich

Copper, foil, ≥99.8% (complexometric)

357456

Sigma-Aldrich

Copper, powder (dendritic), <45 μm, 99.7% 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

223409

Sigma-Aldrich

Copper, shot, −3-+14 mesh, 99%

266744

Sigma-Aldrich

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

254177

Sigma-Aldrich

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

GF66556491

Copper, mesh, 100x100mm, nominal aperture 0.14mm, thickness 0.25mm, wire diameter 0.115mm, 100x100 wires/inch, open area 30.3%, plain weave mesh

GF00920898

Copper, rod, 100mm, diameter 9.5mm, hard, 99.9%

349224

Sigma-Aldrich

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

GF37244729

Copper, tube, 100mm, outside diameter 3.5mm, inside diameter 1.56mm, wall thickness 0.97mm, as drawn, 99.9%

GF28976226

Copper, tube, 500mm, outside diameter 6.35mm, inside diameter 4.93mm, wall thickness 0.71mm, as drawn, 99.9%

326488

Sigma-Aldrich

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

GF16694006

Copper, rod, 50mm, diameter 6.35mm, as drawn, 99.999%

311405

Sigma-Aldrich

Copper, ACS reagent, granular, 10-40 mesh, ≥99.90%

GF98886920

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

GF53493745

Copper - O.F.H.C., sheet, 150x150mm, thickness 3.0mm, 99.95+%

GF50190027

Copper, rod, 1000mm, diameter 4.8mm, hard, 99.9%

GF53876256

Copper, rod, 200mm, diameter 8.0mm, as drawn, 99.99+%