Researchers from the Bellvitge Biomedical Research Institute (IDIBELL) at the University of Barcelona (UB) have coordinated a research into how the IDPN nitrile causes neurological syndromes similar to those of the amyotrophic lateral sclerosis (ALS), a severe neuromuscular degenerative disease. The study, led by Jordi Llorens, has been recently published in Neuropathology and Applied Neurobiology journal.
Nitriles, chemical compounds containing the cyano (-CN) group, are ubiquitous in nature and have diverse applications in industry. In nature they appear as cyanogenic glycosides, for example in bitter almonds, and as aminonitriles, in some legumes. In industry they are used as solvents and intermediates in the synthesis of plastics, synthetic fibers, resins and pharmaceutical products, among others. The consumption by humans or animals of certain nitriles can cause symptoms similar to cyanide poisoning. This fact suggests that the release of this compound is responsible for acute intoxication. Some nitriles realease less cyanide or do it more slowly, causing neurotoxicity and neurological syndromes.
In a variety of diseases of the nervous system appears abnormal accumulation of neurofilaments, fibers that confer stiffness to neurons. Specifically, in amyotrophic lateral sclerosis (ALS) are observed protrusions formed by a large number of neurofilaments in the axons of motor neurons.
In the study, researchers have observed accumulations of neurofilaments strikingly similar to those occurring in ALS, in laboratory rats exposed to IDPN (3.3'-iminodipropionitril). Because of this similarity, the researchers have studied the effect of IDPN to understand the biology of neurofilaments in ALS disease. The disease and the IDPN poisoning cause axonopathy, an injury to axons. The novelty of this study is the observation that the axonopathy causes a marked loss of neurofilaments in the terminals of motor neurons. This observation is relevant because the refraction of the terminals is the critical factor in the degeneration of the motor neuron.