Sahara dust is frequently emitted into the Mediterranean atmosphere and transported by the winds sometimes as far north as central Europe and Great Britain.Saharan dust storms have increased approximately 10-fold during the half-century since the 1950s, causing topsoil loss in Niger, Chad, northern Nigeria, and Burkina Faso. In Mauritania there were just two dust storms a year in the early 1960s; there are about 80 a year since 2007, according to English geographer Andrew Goudie, professor at the University of Oxford. Levels of Saharan dust coming off the east coast of Africa in June 2007 were five times those observed in June 2006, and were the highest observed since at least 1999, which may have cooled Atlantic waters enough to slightly reduce hurricane activity in late 2007. Dust storms have also been shown to increase the spread of disease across the globe. Virus spores in the ground are blown into the atmosphere by the storms with the minute particles and interact with urban air pollution.Short-term effects of exposure to desert dust include immediate increased symptoms and worsening of the lung function in individuals with asthma, increased mortality and morbidity from long-transported dust from both Saharan and Asian dust storms suggesting that long-transported dust storm particles adversely affects the circulatory system. Dust pneumonia is the result of large amounts of dust being inhaled.
People survive owing to low amounts of the SMN protein produced from the SMN2 gene. Nusinersen modulates alternative splicing of the SMN2 gene, functionally converting it into SMN1 gene, thus increasing the level of SMN protein in the CNS.The drug distributes to CNS and peripheral tissues.The half-life is estimated to be 135 to 177 days in cerebrospinal fluid (CSF) and 63 to 87 days in blood plasma. The drug is metabolized via exonuclease (3′- and 5′)-mediated hydrolysis and does not interact with CYP450 enzymes. The primary route of elimination is likely by urinary excretion for nusinersen and its metabolites. Chemistry
Nusinersen is an antisense oligonucleotide in which the 2-hydroxy groups of the ribofuranosyl rings are replaced with 2-O-2-methoxyethyl groups and the phosphate linkages are replaced with phosphorothioate linkages. History
Nusinersen was developed in a collaboration between Adrian Krainer at Cold Spring Harbor Laboratory and Ionis Pharmaceuticals (formerly called Isis Pharmaceuticals). Initial work of target discovery of nusinersen was done by Dr. Ravindra Singh and co-workers at the University of Massachusetts Medical School funded by Cure SMA.Starting in 2012, Ionis partnered with Biogen on development and, in 2015, Biogen acquired an exclusive license to the drug for a US$75 million license fee, milestone payments up to US$150 million, and tiered royalties thereafter; Biogen also paid the costs of development subsequent to taking the license.