Diacetylbis(N(4)-methylthiosemicarbazonato) Copper(II) (CuII(atsm)) Protects against Peroxynitrite-induced Nitrosative Damage and Prolongs Survival in Amyotrophic Lateral Sclerosis Mouse Model

Soon, Cynthia P.W.; Donnelly, Paul S.; Turner, Bradley J.; Hung, Lin W.; Crouch, Peter J.; Sherratt, Nicki A.; Tan, Jiang-Li; Lim, Nastasia K.-H.; Lam, Linh Q; Bica, Laura; Lim, SinChun; Hickey, James L.; Morizzi, Julia; Powell, Andrew K.; Finkelstein, David I.; Culvenor, Janetta G.; Masters, Colin L.; Duce, James A.; White, Anthony R.; Barnham, Kevin J.; Li, Qiao‐Xin

doi:10.1074/jbc.m111.274407

Cited by 126 publications

(181 citation statements)

References 48 publications

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“…Furthermore, the prevention of phosphorylated and fragmented TDP-43 aggregation by copper has also been confirmed in the mutant TDP-43 (A315 T) transgenic mouse (Ref. 104).…”

Section: Copper Homeostasis and Mitochondrial Dysfunctionmentioning

confidence: 80%

“…Cu II (atsm), which transports Cu(II) throughout the body and delivers it when it reaches oxidative tissues, has shown a therapeutic effect in several animal models of ALS and PD (Refs 52,63,104,(106)(107)(108). In particular, Cu II (atsm) significantly improves locomotor function and increases mouse survival in SOD1-G93A, SOD1-G37R (Refs 52, 104, 107, 108) and TDP-43 (A315 T) mouse models of ALS (Ref.…”

Section: Copper Homeostasis and Mitochondrial Dysfunctionmentioning

confidence: 99%

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Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis

Gil-Bea

Aldanondo

Munáin

et al. 2017

Expert Rev. Mol. Med.

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Amyotrophic lateral sclerosis (ALS) is a severe neuromuscular disease characterised by a progressive loss of motor neurons that usually results in paralysis and death within 2 to 5 years after disease onset. The pathophysiological mechanisms involved in ALS remain largely unknown and to date there is no effective treatment for this disease. Here, we review clinical and experimental evidence suggesting that dysregulation of copper homeostasis in the central nervous system is a crucial underlying event in motor neuron degeneration and ALS pathophysiology. We also review and discuss novel approaches seeking to target copper delivery to treat ALS. These novel approaches may be clinically relevant not only for ALS but also for other neurological disorders with abnormal copper homeostasis, such as Parkinson's, Huntington's and Prion diseases.

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“…Furthermore, the prevention of phosphorylated and fragmented TDP-43 aggregation by copper has also been confirmed in the mutant TDP-43 (A315 T) transgenic mouse (Ref. 104).…”

Section: Copper Homeostasis and Mitochondrial Dysfunctionmentioning

confidence: 80%

Section: Copper Homeostasis and Mitochondrial Dysfunctionmentioning

confidence: 99%

Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis

Gil-Bea

Aldanondo

Munáin

et al. 2017

Expert Rev. Mol. Med.

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“…2A). ATSM has been developed for tumor imaging and diagnosis and is currently in clinical trials (13)(14)(15). In the absence of copper, ATSM at concentrations of up to 10 M had no apparent inhibitory effect on M. tuberculosis.…”

mentioning

confidence: 99%

Copper-Boosting Compounds: a Novel Concept for Antimycobacterial Drug Discovery

Speer

Shrestha

Bossmann

et al. 2013

Antimicrob Agents Chemother

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“…First, animals co-expressing human ''copper chaperone for SOD1'' (CCS) have copper deficient mutant SOD1 and markedly accelerated disease progression (15). Second, treatment with CuATSM, which specifically releases copper into cells that have a defective electron transport chain, slows mouse disease progression (3,(16)(17)(18)(19). Finally, treatment with chelators that lower spinal cord copper levels can also slow mouse disease progression (9).…”

Section: Mechanismsmentioning

confidence: 99%

“…We found several studies evaluating the effect of CuATSM on mutant SOD1 mice with and without CCS coexpression (3,(16)(17)(18)(19). These are summarized in Table 3.…”

Section: Preclinical Datamentioning

confidence: 99%