Peroxynitriitin ja proteiininitraation vaaroista (ALS, Sinkkivajeinen SOD)

Kari A. Trumbull , Joseph   S. Beckman, A role of Cu in the toxicity of Zn-deficient superoxidedismutase (SOD) to Motor neuron in ALS (2009).
 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2842582/

 Sinkkivajeinen SOD entsyymi   vaikuttaa kuparitoksisesti neuroneihin  ALS taudissa.

Proposed mechanism for the role of Zinc deficient SOD1 in the formation of peroxynitrite, leading to protein nitration and motor neuron apoptosis. reduced Cu in Zinc-deficient SOD1 catalyses the one-electron reduction of oxygen to superoxide anion radical. Nitric oxide(NO) reacts with superoxide anion radical  at a diffusion limited rate to form peroxynitrite.
Zn-deficient SOD1 will also catalyze the nitration of proteins such as neurofilaments. Nitration of neurofilaments interferes with the assembly, and disassembled neurofilaments have a particularly high affinity for Zn that can potentially sequester  Zn from  SOD1. Tyrosine nitration by peroxynitrite has now been strongly implicated in promoting motor neuron apoptosis and making astrocytes reactive. reactive astrocytes also contribute to motor neuron death and might participate in the progressive death of motor neurons.