|1.||Loh, Stewart N: 2 articles (11/2015 - 07/2010)|
|2.||Leary, Scot C: 2 articles (06/2015 - 09/2006)|
|3.||Tsivkovskii, Ruslan: 2 articles (04/2003 - 08/2002)|
|4.||Lutsenko, Svetlana: 2 articles (04/2003 - 08/2002)|
|5.||Walker, Joel M: 2 articles (04/2003 - 08/2002)|
|6.||Blanden, Adam R: 1 article (11/2015)|
|7.||Yu, Xin: 1 article (11/2015)|
|8.||Carpizo, Darren R: 1 article (11/2015)|
|9.||Levine, Arnold J: 1 article (11/2015)|
|10.||Pacheu-Grau, David: 1 article (06/2015)|
|1.||Hepatolenticular Degeneration (Wilson's Disease)
04/21/2008 - "To analyze the metallochaperone antioxidant-1 (Atox1) gene sequence in Wilson disease patients. "
04/01/2003 - "Functional properties of the human copper-transporting ATPase ATP7B (the Wilson's disease protein) and regulation by metallochaperone Atox1."
08/02/2002 - "Metallochaperone Atox1 transfers copper to the NH2-terminal domain of the Wilson's disease protein and regulates its catalytic activity."
09/01/2000 - "The Hah1 metallochaperone protein is implicated in copper delivery to the Menkes and Wilson disease proteins. "
09/01/2000 - "Structural basis for copper transfer by the metallochaperone for the Menkes/Wilson disease proteins."
11/01/2015 - "Reactivating mutant p53 using small molecules as zinc metallochaperones: awakening a sleeping giant in cancer."
07/01/2010 - "This review discusses mechanisms by which zinc modulates folding and misfolding of p53, how improper metal binding and release leads to loss of function and cancer, and how misfolding can be rescued by metallochaperones."
04/01/2013 - "SCO2, a cytochrome c oxidase assembly factor, is a metallochaperone which is involved in the biogenesis of cytochrome c oxidase subunit II. Here we have shown that SCO2 functions as an apoptotic protein in tumor xenografts, thus providing an alternative pathway for p53-mediated apoptosis. "
|3.||Hypertrophic Cardiomyopathy (Asymmetric Septal Hypertrophy)
09/01/2006 - "Mutations in SCO2, a metallochaperone involved in mitochondrial copper delivery, are associated with early onset, fatal hypertrophic cardiomyopathy. "
05/01/2009 - "Here, we analyzed the impact on CcO assembly and tissue copper levels of a G132S mutation in the juxtamembrane region of SCO1 metallochaperone associated with early onset hypertrophic cardiomyopathy, encephalopathy, hypotonia, and hepatopathy, assessed the total copper content of various SURF1 and SCO2-deficient tissues, and investigated the possible physical association between CcO and Sco1. "
01/01/2007 - "Improperly folded metal cofactor-containing proteins (e.g., copper chaperone for superoxide dismutase, CCS) are believed to play a key role in several protein-misfolding diseases (e.g., Alzheimer's disease or Amyotrophic Lateral Sclerosis) because under regular physiological conditions, metallochaperones activate or stabilize the native conformation of important metalloproteins (e.g., superoxide dismutase) in certain cellular processes. "
|3.||Proteins (Proteins, Gene)
|7.||Electron Transport Complex IV (Cytochrome c Oxidase)
|8.||cytochrome C oxidase subunit II
|1.||Heterologous Transplantation (Xenotransplantation)