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The loss of stability of a naturally occurring ATP7A mutant is the cause of Menkes disease
Authors
Banci, L., Bertini, I., Cantini, F., Migliardi, M., Rosato, A., Wang, S.
Assembly
Copper-transporting ATPase 1(E.C.3.6.3.4)
Entity
1. Copper-transporting ATPase 1(E.C.3.6.3.4) (polymer, Thiol state: free and other bound), 75 monomers, 8179.493 Da Detail

MGDGVLELVV RGMTCASCVH KIESSLTKHR GILYCSVALA TNKAHIKYDP EIIGPRDIIH TIESLGFEAS LVKIE


2. CU1 (non-polymer), 63.546 Da
Total weight
8243.039 Da
Max. entity weight
8179.493 Da
Entity Connection
na 2 Detail

IDTypeValue orderAtom ID 1Atom ID 2
1nasing1:CYS15:SG2:CU11:CU
2nasing1:CYS18:SG2:CU11:CU

Source organism
Homo sapiens
Exptl. method
NMR
Refine. method
torsion angle dynamics coupled with simulated annealing followed by restrained energy minimization
Data set
assigned_chemical_shifts
Chem. Shift Complete
Sequence coverage: 97.3 %, Completeness: 87.6 %, Completeness (bb): 94.7 % Detail

Polymer type: polypeptide(L)

Total1H15N
All87.6 % (446 of 509)86.4 % (376 of 435)94.6 % (70 of 74)
Backbone94.7 % (215 of 227)94.8 % (146 of 154)94.5 % (69 of 73)
Sidechain81.9 % (231 of 282)81.9 % (230 of 281)100.0 % (1 of 1)
Aromatic52.4 % (11 of 21)52.4 % (11 of 21)
Methyl92.5 % (49 of 53)92.5 % (49 of 53)

1. Copper-transporting ATPase 1(E.C.3.6.3.4)

MGDGVLELVV RGMTCASCVH KIESSLTKHR GILYCSVALA TNKAHIKYDP EIIGPRDIIH TIESLGFEAS LVKIE

Sample

Pressure 1 atm, Temperature 298 K, pH 7


#NameIsotope labelingTypeConcentration
1Copper-transporting ATPase 1(E.C.3.6.3.4)[U-15N]0.8 mM
2DTT5 mM
3phosphate buffer100 mM
4H2O90 %
5D2O10 %

Protein Blocks Logo
Calculated from 30 models in PDB: 1YJV, Strand ID: A Detail


Release date
2005-11-13
Citation
An atomic-level investigation of the disease-causing A629P mutant of the Menkes protein, ATP7A
Banci, L., Bertini, I., Cantini, F., Migliardi, M., Rosato, A., Wang, S.
J. Mol. Biol. (2005), 352, 409-417, PubMed 16083905 , DOI 10.1016/j.jmb.2005.07.034 ,
Related entities 1. Copper-transporting ATPase 1(E.C.3.6.3.4), : 1 : 1 : 4 : 356 entities Detail
Interaction partners 1. Copper-transporting ATPase 1(E.C.3.6.3.4), : 5 interactors Detail
Experiments performed 8 experiments Detail
nullKeywords copper(I), metal homeostasis, metallochaperone, protein-protein interaction