Blood-Brain Barriers (Bbb)

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Blood brain barriers Blood-brain barriers (BBB) are unique structural features of capillaries found in the brain that are responsible for making it difficult the diffusion of substances from the capillary blood into the brain compared to similar capillary diffusion elsewhere in the body (Nag, 2003). The BBB have tighter barrier between their endothelial cells lining brain capillaries in comparison to those lining capillaries elsewhere in the body. The capillary endothelium of the BBB has thicker basement membrane relative to other capillaries in other body parts. Unlike other capillaries, the BBB are also shielded by astrocytes. Blood brain barriers thus play a crucial role in maintaining homeostasis in the brain. The endothelial cells serve the function of restricting diffusion of microscopic materials such as bacteria and hydrophilic or large molecules into the cerebrospinal fluid (CSF) on the one hand, while permitting passage of micro hydrophobic molecules e.g. carbon dioxide, oxygen, alcohols, steroids (Nag, 2003). Diffusion of water-soluble molecules such as chloride ions, potassium, and sodium through the BBB is facilitated by special carrier molecules. Lesch-Nyhan syndrome Lesch-Nyhan syndrome, also referred to as Kelley-Seegmiller syndrome or HPRT deficiency refers to a rare genetic disorder caused by the deficiency of hypoxanthine-guanine phosphoribosyltransferase (HGPRT) enzyme, which is produced through mutations in the HPRT gene found on the X chromosome. HPRT gene serves to catalyze the reaction needed to check the buildup of uric acid (which is a nitrogenous waste product ordinarily excreted from the body via the kidneys) (Gillberg, 2003). When the HPRT gene severely mutates, there is lack of HPRT enzyme action which results in heightened uric acid levels in the blood (a condition known as hyperuricemia) as a result of massive oversynthesis of
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