2ga7

File:2ga7.gif

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Solution structure of the copper(I) form of the third metal-binding domain of ATP7A protein (menkes disease protein)

OverviewOverview

The third metal-binding domain of the human Menkes protein (MNK3), a, copper(I)-transporting ATPase, has been expressed in Escherichia coli and, characterized in solution. The solution structure of MNK3, its, copper(I)-binding properties, and its interaction with the physiological, partner, HAH1, have been studied. MNK3 is the domain most dissimilar in, structure from the other domains of the Menkes protein. This is reflected, in a significant rearrangement of the last strand of the four-stranded, beta-sheet when compared with the other known homologous proteins or, protein domains. MNK3 is also peculiar with respect to its interaction, with the copper(I) ion, as it was found to be a comparatively weak binder., Copper(I) transfer from metal-loaded HAH1 was observed experimentally, but, the metal distribution was shifted toward binding by HAH1. This is at, variance with what is observed for the other Menkes domains.

DiseaseDisease

Known diseases associated with this structure: Analgesia from kappa-opioid receptor agonist, female-specific OMIM:[155555], Blond/light brown hair and/or fair skin OMIM:[155555], Cutis laxa, neonatal OMIM:[300011], Melanoma, susceptiblity to OMIM:[155555], Menkes disease OMIM:[300011], Occipital horn syndrome OMIM:[300011], Oculocutaneous albinism, type II, modifier of OMIM:[155555], Red hair/fair skin OMIM:[155555], UV-induced skin damage, susceptibility to OMIM:[155555]

About this StructureAbout this Structure

2GA7 is a Single protein structure of sequence from Homo sapiens with CU1 as ligand. Active as Copper-exporting ATPase, with EC number 3.6.3.4 Full crystallographic information is available from OCA.

ReferenceReference

Solution structure and intermolecular interactions of the third metal-binding domain of ATP7A, the Menkes disease protein., Banci L, Bertini I, Cantini F, DellaMalva N, Herrmann T, Rosato A, Wuthrich K, J Biol Chem. 2006 Sep 29;281(39):29141-7. Epub 2006 Jul 26. PMID:16873374

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