The MC system is a family of five G protein-coupled receptors, melanocortin-1 through -5 receptors (MC1R-MC5R), that play an integral role in neuroendocrine signaling ( Figure 1 , particularly in centers of metabolic control including the arcuate nucleus (ARC), paraventricular nucleus (PVN), nucleus of the solitary tract (NTS), and paraventricular thalamus (PVT) ( Figure 1 The melanocortin receptors are modulated by several endogenous peptides, such as -, -, and -melanocyte stimulating hormone (MSH) and adrenocorticotropic hormone (ACTH), which are derived from proopiomelanocortin (POMC) precursors ( Early prioritization of MC4R as a therapeutic target for obesity was driven by converging genetic and pharmacological evidence demonstrating its primary role in regulating food intake and body weight
Neurologically, ALD/AMN can manifest with highly variable phenotypes, ranging from very aggressive and rapidly fatal inflammatory CNS demyelination in young boys to a more slowly progressive adult form either with cerebral white matter inflammatory lesions, or a more indolent progressive myelopathy and peripheral neuropathy (Moser, 1997)
JPE, Macdonald AMC
Cell Death and Stress Cell death can result from different pathways, and oxidative stress can be an upstream trigger
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