1q8l
From Proteopedia
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Second Metal Binding Domain of the Menkes ATPase
Contents |
Overview
Biological utilisation of copper requires that the metal, in its ionic, forms, be meticulously transported, inserted into enzymes and regulatory, proteins, and excess be excreted. To understand the trafficking process, it is crucial that the structures of the proteins involved in the varied, processes be resolved. To investigate copper binding to a family of, structurally related copper-binding proteins, we have characterised the, second Menkes N-terminal domain (MNKr2). The structure, determined using, 1H and 15N heteronuclear NMR, of the reduced form of MNKr2 has revealed, two alpha-helices lying over a single beta-sheet and shows that the, binding site, a Cys(X)2Cys pair, is located on an exposed loop. 1H-15N, HSQC experiments demonstrate that binding of Cu(I) causes changes that are, localised to conserved residues adjacent to the metal binding site., Residues in this area are important to the delivery of copper by the, structurally related Cu(I) chaperones. Complementary site-directed, mutagenesis of the adjacent residues has been used to probe the structural, roles of conserved residues.
Disease
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 Structure
1Q8L is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Structure and metal binding studies of the second copper binding domain of the Menkes ATPase., Jones CE, Daly NL, Cobine PA, Craik DJ, Dameron CT, J Struct Biol. 2003 Sep;143(3):209-18. PMID:14572476
Page seeded by OCA on Mon Nov 12 18:51:33 2007
