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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: all free), 75 monomers, 8205.530 Da Detail

MGDGVLELVV RGMTCASCVH KIESSLTKHR GILYCSVALA TNKAHIKYDP EIIGPRDIIH TIESLGFEPS LVKIE


Formula weight
8205.53 Da
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: 81.6 %, Completeness (bb): 93.3 % Detail

Polymer type: polypeptide(L)

Total1H15N
All81.6 % (418 of 512)79.7 % (350 of 439)93.2 % (68 of 73)
Backbone93.3 % (210 of 225)93.5 % (143 of 153)93.1 % (67 of 72)
Sidechain72.5 % (208 of 287)72.4 % (207 of 286)100.0 % (1 of 1)
Aromatic57.1 % (12 of 21)57.1 % (12 of 21)
Methyl98.1 % (51 of 52)98.1 % (51 of 52)

1. Copper-transporting ATPase 1

MGDGVLELVV RGMTCASCVH KIESSLTKHR GILYCSVALA TNKAHIKYDP EIIGPRDIIH TIESLGFEPS LVKIE

Sample

Pressure 1 atm, Temperature 298 K, pH 7


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

Protein Blocks Logo
Calculated from 30 models in PDB: 1YJR, 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 ,
Entries sharing articles BMRB: 3 entries Detail
  BMRB: 6480 released on 2005-11-13
    Title The loss of stability of a naturally occurring ATP7A mutant is the cause of Menkes disease
  BMRB: 6481 released on 2005-11-13
    Title The loss of stability of a naturally occurring ATP7A mutant is the cause of Menkes disease
  BMRB: 6482 released on 2005-11-13
    Title The loss of stability of a naturally occurring ATP7A mutant is the cause of Menkes disease
Related entities 1. Copper-transporting ATPase 1(E.C.3.6.3.4), : 1 : 1 : 4 : 346 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