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Translational research investigations on ATP7A: an important human copper ATPase.

Translational research investigations on ATP7A: an important human copper ATPase.

Annals of the New York Academy of Sciences (2014-04-17)

Stephen G Kaler

ABSTRACT

In more than 40 years since copper deficiency was delineated in pediatric subjects with Menkes disease, remarkable advances in our understanding of the clinical, biochemical, and molecular aspects of the human copper transporter ATP7A have emerged. Mutations in the gene encoding this multitasking molecule are now implicated in at least two other distinctive phenotypes: occipital horn syndrome and ATP7A-related isolated distal motor neuropathy. Several other novel inherited disorders of copper metabolism have been identified in the past several years, aided by advances in human gene mapping and automated DNA sequencing. In this paper, I review the history and evolution of our understanding of disorders caused by impaired ATP7A function, and outline future challenges.

MATERIALS

Product Number

Brand

Product Description

Sigma-Aldrich

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

Sigma-Aldrich

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

Sigma-Aldrich

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

Sigma-Aldrich

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

Sigma-Aldrich

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

Sigma-Aldrich

Copper, wire, diam. 1.0 mm, ≥99.9%

Sigma-Aldrich

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

Sigma-Aldrich

Copper, turnings, purum p.a., ≥99.0%

Sigma-Aldrich

Copper, foil, ≥99.8% (complexometric)

Sigma-Aldrich

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

Sigma-Aldrich

Copper, powder, <75 μm, 99%

Sigma-Aldrich

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

Copper, wire reel, 500m, diameter 0.15mm, annealed, silver plated, 99.9%

Copper, wire reel, 250m, diameter 0.04mm, annealed, 99.9%

Copper, wire reel, 1000m, diameter 0.075mm, annealed, 99.9%

Copper, wire reel, 1m, diameter 1.0mm, as drawn, 99.99+%

Copper, wire reel, 20m, diameter 1.0mm, as drawn, 99.98+%

Copper, wire reel, 100m, diameter 0.15mm, annealed, silver plated, 99.9%

Copper, wire reel, 1000m, diameter 0.05mm, annealed, 99.9%

Copper, wire reel, 0.2m, diameter 0.1mm, aluminum cladding, outside diameter 0.25mm, 99.9%

Copper, wire reel, 500m, diameter 0.1mm, annealed, 99.9%

Copper, wire reel, 1000m, diameter 0.125mm, annealed, 99.9%

Copper, wire reel, 200m, diameter 0.15mm, annealed, silver plated, 99.9%

Copper, wire reel, 10m, diameter 1.0mm, annealed, 99.9%

Copper, wire reel, 0.5m, diameter 0.125mm, as drawn, 99.99+%

Copper, wire reel, 500m, diameter 0.25mm, annealed, 99.9%

Copper, wire reel, 50m, diameter 0.75mm, as drawn, 99.98+%

Copper, wire reel, 2000m, diameter 0.05mm, annealed, 99.9%

Copper, wire reel, 200m, diameter 0.5mm, annealed, silver plated, 99.9%

Copper, wire reel, 5m, diameter 0.025mm, as drawn, 99.99+%