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This coordinated suppression subverts macrophage defenses

H.Bjrke-MonsenA.-L.BritoA.GuantJ.-L.MillerJ

Severe cases of homocystinuria are typically caused by autosomal recessive genetic defects and exhibit characteristic clinical features including ocular lens dislocation, marfanoid features and other skeletal abnormalities including osteoporosis, intellectual disability, and thromboembolic disease, the latter frequently being the cause of premature death in affected individuals (28)

In addition to KEAP1, other forms of regulatory proteins of NRF-2 have recently been discovered, such as the E3-Ubiquitin-ligase adaptor, and the -transducin repeats-containing protein (-TrCP), which can also be degraded by Glycogen Synthase Kinase 3 (GSK-3), phosphorylizing NRF-2 into its residue as serine in the Neh 6 domain, corresponding to the recognition of -TrCP [18] (Fig