Distinct Functional Roles for the Menkes and Wilson Copper Translocating P-type ATPases in Human Placental Cells

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Hormonal regulation of the Menkes and Wilson copper-transporting ATPases in human placental Jeg-3 cells.

Copper deficiency during pregnancy results in early embryonic death and foetal structural abnormalities including skeletal, pulmonary and cardiovascular defects. During pregnancy, copper is transported from the maternal circulation to the foetus by mechanisms which have not been clearly elucidated. Two copper-transporting ATPases, Menkes (ATP7A; MNK) and Wilson (ATP7B; WND), are expressed in th...

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The copper-transporting ATPases, menkes and wilson disease proteins, have distinct roles in adult and developing cerebellum.

Copper is essential for brain metabolism, serving as a cofactor to superoxide dismutase, dopamine-beta-hydroxylase, amyloid precursor protein, ceruloplasmin, and other proteins required for normal brain function. The copper-transporting ATPases ATP7A and ATP7B play a central role in distribution of copper in the central nervous system; genetic mutations in ATP7A and ATP7B lead to severe neurode...

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Biochemical characterization of P-type copper ATPases

Copper ATPases, in analogy with other members of the P-ATPase superfamily, contain a catalytic headpiece including an aspartate residue reacting with ATP to form a phosphoenzyme intermediate, and transmembrane helices containing cation-binding sites [TMBS (transmembrane metal-binding sites)] for catalytic activation and cation translocation. Following phosphoenzyme formation by utilization of A...

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FUNCTIONAL SIGNIFICANCE OF THE -SUBUNIT FOR HETERODIMERIC P-TYPE ATPases

We have reviewed the structural and functional role of the -subunit in a subfamily of the P-ATPases known as the / -heterodimeric, cation-exchange ATPases. The subfamily consists of the various isoforms of Na+/K+ATPase and H+/K+-ATPase, both of which pump a cation out of the cell (Na+ or H+, respectively) in recycle exchange for K+. Much of the earlier work has emphasized the functional activit...

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Copper-dependent interaction of glutaredoxin with the N termini of the copper-ATPases (ATP7A and ATP7B) defective in Menkes and Wilson diseases.

The P-type ATPases affected in Menkes and Wilson diseases, ATP7A and ATP7B, respectively, are key copper transporters that regulate copper homeostasis. The N termini of these proteins are critical in regulating their function and activity, and contain six copper-binding motifs MxCxxC. In this study, we describe the identification of glutaredoxin (GRX1) as an interacting partner of both ATP7A an...

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ژورنال

عنوان ژورنال: Cellular Physiology and Biochemistry

سال: 2007

ISSN: 1015-8987,1421-9778

DOI: 10.1159/000110718