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• W2092245845 endingPage "4250" @default.
• W2092245845 startingPage "4242" @default.
• W2092245845 abstract "Cu,Zn-superoxide dismutase (SOD1) is a cytosolic antioxidant enzyme, and its mutation has been implicated in amyotrophic lateral sclerosis (ALS), a disease causing a progressive loss of motor neurons. Although the pathogenic mechanism of ALS remains unclear, it is hypothesized that some toxic properties acquired by mutant SOD1 play a role in the development of ALS. We have examined the structural and catalytic properties of an ALS-linked mutant of human SOD1, His43Arg (H43R), which is characterized by rapid disease progression. As revealed by circular dichroism spectroscopy, H43R assumes a stable β-barrel structure in the Cu(2+),Zn(2+)-bound holo form, but its metal-depleted apo form is highly unstable and readily unfolds or misfolds into an irregular structure at physiological temperature. The conformational change occurs as a two-state transition from a nativelike apo form to a denatured apo form with a half-life of ∼0.5 h. At the same time as the denaturation, the apo form of H43R acquires pro-oxidant potential, which is fully expressed in the presence of Cu(2+) and H(2)O(2), as monitored with a fluorogenic probe for detecting pro-oxidant activity. Comparison of d-d absorption bands suggests that the Cu(2+) binding mode of the denatured apo form is different from that of the native holo form. The denatured apo form of H43R is likely to provide non-native Cu(2+) binding sites where the Cu(2+) ion is activated to catalyze harmful oxidation reactions. This study raises the possibility that the structural instability and the resultant Cu-dependent pro-oxidant activity of the apo form of mutant SOD1 may be one of the pathogenic mechanisms of ALS." @default.
• W2092245845 created "2016-06-24" @default.
• W2092245845 creator A5025816894 @default.
• W2092245845 creator A5031566254 @default.
• W2092245845 creator A5052552300 @default.
• W2092245845 creator A5067165248 @default.
• W2092245845 creator A5075339712 @default.
• W2092245845 date "2011-05-02" @default.
• W2092245845 modified "2023-10-16" @default.
• W2092245845 title "Structural Instability and Cu-Dependent Pro-Oxidant Activity Acquired by the Apo Form of Mutant SOD1 Associated with Amyotrophic Lateral Sclerosis" @default.
• W2092245845 cites W1940436875 @default.
• W2092245845 cites W1967469285 @default.
• W2092245845 cites W1969665448 @default.
• W2092245845 cites W1980497908 @default.
• W2092245845 cites W1981120411 @default.
• W2092245845 cites W1986438501 @default.
• W2092245845 cites W1988054144 @default.
• W2092245845 cites W1989270637 @default.
• W2092245845 cites W1989403800 @default.
• W2092245845 cites W1991039509 @default.
• W2092245845 cites W1995075942 @default.
• W2092245845 cites W1999913981 @default.
• W2092245845 cites W2004978936 @default.
• W2092245845 cites W2005545438 @default.
• W2092245845 cites W2009030515 @default.
• W2092245845 cites W2010716981 @default.
• W2092245845 cites W2011766911 @default.
• W2092245845 cites W2013135033 @default.
• W2092245845 cites W2020158404 @default.
• W2092245845 cites W2022367303 @default.
• W2092245845 cites W2024345302 @default.
• W2092245845 cites W2027426714 @default.
• W2092245845 cites W2029363013 @default.
• W2092245845 cites W2030817463 @default.
• W2092245845 cites W2032670789 @default.
• W2092245845 cites W2032881765 @default.
• W2092245845 cites W2034370881 @default.
• W2092245845 cites W2034949213 @default.
• W2092245845 cites W2035689722 @default.
• W2092245845 cites W2037315437 @default.
• W2092245845 cites W2041933318 @default.
• W2092245845 cites W2044118737 @default.
• W2092245845 cites W2044780988 @default.
• W2092245845 cites W2051401615 @default.
• W2092245845 cites W2053712580 @default.
• W2092245845 cites W2055307767 @default.
• W2092245845 cites W2064919114 @default.
• W2092245845 cites W2067279542 @default.
• W2092245845 cites W2068876170 @default.
• W2092245845 cites W2070458959 @default.
• W2092245845 cites W2072874326 @default.
• W2092245845 cites W2073864172 @default.
• W2092245845 cites W2074722508 @default.
• W2092245845 cites W2082066163 @default.
• W2092245845 cites W2083339401 @default.
• W2092245845 cites W2084450708 @default.
• W2092245845 cites W2091436287 @default.
• W2092245845 cites W2095360134 @default.
• W2092245845 cites W2096386031 @default.
• W2092245845 cites W2096678032 @default.
• W2092245845 cites W2099071260 @default.
• W2092245845 cites W2101119366 @default.
• W2092245845 cites W2101422765 @default.
• W2092245845 cites W2105112194 @default.
• W2092245845 cites W2105442888 @default.
• W2092245845 cites W2107768418 @default.
• W2092245845 cites W2112761293 @default.
• W2092245845 cites W2123413118 @default.
• W2092245845 cites W2123692189 @default.
• W2092245845 cites W2123779560 @default.
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• W2092245845 cites W2127248737 @default.
• W2092245845 cites W2132193523 @default.
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• W2092245845 cites W2166155593 @default.
• W2092245845 cites W4236884331 @default.
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• W2092245845 doi "https://doi.org/10.1021/bi200338h" @default.
• W2092245845 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/21506602" @default.
• W2092245845 hasPublicationYear "2011" @default.
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