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Tuberous sclerosis complex was rst recognized as a syndrome by D sir -Magloire Bourneville in 1880; this was followed by a series of reports with similar characteristics (Riccio & Harrison, 1998). Tuberous sclerosis is a neurocutaneous disorder characterized by esh-colored discrete marks that may resemble acne (adenoma sebaceum) and typically occur in a butter y distribution in the folds of skin around the nasal area. It was named to re ect the potatolike lesions in the cerebral cortex that result in hard areas of tissues called cortical tubers (Hunt, 2006; Roach, 1992). In addition to the adenoma sebaceum, there may be areas where there is an absence of pigmentation (amelanotic nevus), most often on the trunk and limbs (Chalhub, 1976). Tuberous sclerosis complex involves multiple systems and abnormal growth of a variety of organs, including the skin, kidneys, and central nervous system (Hunt, 2006). Tuberous sclerosis is an inherited autosomal dominant disorder (Roach, 1992); however, it also may occur as a genetic mutation with no family history. The gene location is believed to be at 9q34 and 11q23 (Connor, 1990). There are also some indications of involvement of chromosome 16 as well as complex genetic interactions (Zaroff, Devinsky, Miles, & Ban, 2004). In its more severe form, tuberous sclerosis complex, mutations are in the tumor suppressor genes, TSC1 (Hamartin) or TSC2 (Tuberin) (Curatolo, D Argenzio, Cerminara, & Bombardieri, 2008; de Vries et al., 2005; L. A. Devlin, Shepherd, Crawford, & Morrison, 2006). These are proteins that form a cytosolic complex that inhibits the mTOR pathway, thus controlling cell growth and proliferation (Curatolo et al., 2008). Pathologically, abnormalities of neuronal migration, cellular differentiation, and excessive cellular proliferation all contribute to the formation of the different brain lesions associated with tuberous sclerosis. asp.net qr code reader QR Code Scanner in ASP.Net - CodeProject
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