Treatment of lead encephalopathy in children

Critical role for Rsk2 in T-lymphocyte activation

“…40 Finally, loss-of-function mutations of p90 Rsk2 in humans cause the Coffin-Lowry syndrome, 41 an X-linked form of mental retardation that is associated with delayed bone age, delayed closure of fontanelles, and short stature. 42,43 We now show that IL-2 and IL-15 rapidly activate Rsk1 and Rsk2 in human T cells. Moreover, our data indicate a nonredundant role for Rsk2 in TCR/cytokine signaling based on expression of various Rsk constructs as well as analysis of Rsk2-deficient mice.…”

The free fatty acid metabolome in cerebral ischemia following human mesenchymal stem cell transplantation in rats

“…Generally, deficiencies in essential PUFA may be caused by an inflammatory reaction involving excessive oxidation, diminished antioxidant defenses, cellular damage, high rates of cell division, and cytokine-mediated reactions [22,33]. In particular, excessive amounts of oxygen radicals stimulate lipid peroxidation and depletion of essential PUFAs, thereby disturbing metabolic pathways during MCAo.…”

“…The n−6 and n−3 PUFAs present in the brain are not synthesized de novo in humans, and must either be obtained from diet or synthesized in the brain from linoleic acid (as the n−6 PUFA precursor) and α-linolenic acid (as the n−3 PUFA precursor) supplied in plasma [22]. In the MCAo group, the increased levels of linoleic acid and arachidonic acid may mediate and regulate inflammation, and this may explain the inflammatory reaction associated with MCAo.…”

“…In addition, linoleic acid found in clinical blood samples may protect against ischemic stroke, possibly by decreasing blood pressure, reducing platelet aggregation, and enhancing the deformability of erythrocytes [17]. However, analyses of differences in FFA composition in serum and in brain [22], and monitoring of biochemical changes mediating the transplantation of hMSC have not yet been performed.…”

Deletion of the Coffin–Lowry Syndrome Gene Rsk2 in Mice is Associated With Impaired Spatial Learning and Reduced Control of Exploratory Behavior

“…Whereas mental disability is the only symptom of the disease in non-syndromic forms, syndromic forms of mental retardation are characterized by a combination of mental disabilities with physical malformations and/or metabolic disturbances. Coffin-Lowry Syndrome (CLS) is a rare syndromic form of mental retardation that shows X-linked inheritance with an estimated incidence of 1 in 50,000-100,000 males (Coffin et al 1966;Lowry et al 1971). It is characterized by moderate to severe psychomotor retardation, growth retardation (short stature), facial and digital dysmorphisms, as well as progressive skeletal deformations (Hanauer and Young 2002).…”