Sfingolipids

Sfingolipids is the main structural components of cellular membranes; the myelin is especially rich with sfingolipids. In a structure of a sfingolipids are similar to phospholipids, but their hydrophilic skeleton is presented not glyceroscrap, and seriny. Makes basis of sfingolipid. Sfingozin is formed in reaction between a palmitoil-Co and seriny in which palmitoil-Koa loses one atom of carbon which is emitted in the form of CO2. At N-acetylation of sfingozin tseramida which are a part of many sfingolipid, for example a sfingomiyelin are formed. Besides, tseramid contains in tserebrozidakh and the gangliozidakh (the connections containing the remains of carbohydrates). Sfingolipidoza are diseases of lysosomes at which degradation of sfingomiyelin is broken. Accumulation of lipids in fabrics leads to development of diseases.

Sfingomiyelin

Sfingomiyelin consists of a tseramid and a fosforilkholin. He is also called tseramidfosforilkholiny. On the structure sfingomielin is similar to fosfatidilkholin. Please pay attention to Pankramin.

Tserebrozida

Tserebrozid is formed when binding a monosaccharide with tseramidy. So, when binding glucose with tseramidy it is formed glukocerebroside (glukozileramide), and when binding galactose galactoziltserebrozid (galactotseramid). Tserebrozida also call "monoglikoziltseramidam". Globozid is tserebrozida containing several carbohydrate remains.

Illness to Gosha

Illness to Gosha which is inherited on autosomal recessively type most often occurs among lysosomic illnesses of accumulation. An etiology a failure of p-glyukotserebrozidazy enzyme (fig. 36.3). It leads to excess accumulation of glucocerebroside in a brain, liver, marrow, a lien. At illness to Gosha I of type (without lesions of a nervous system) fermentozamestitelny therapy is carried out: patients receive recombinant p-glyukotserebrozidazu. Perhaps, the gene therapy based on transfer of a gene of a β-tserebrozidaza in DNA of the hemopoietic stem cells will be used in the future.

Gangliosides and globozida

Gangliosides are formed at linkng of a tseramid with an oligosaccharide and N-atsetilneyraminovoy by acid (sialic acid). Gangliosides make about 5% of all lipids of a brain.

Fabri's illness

Fabri's illness is the infrequent H-linked lysosomic illness at which a failure is observed and-galaktotserebrozidazy A. Eto leads to accumulation in an organism of globozid of tseramidtrigeksozid (globotriaziltseramid). Kidneys and cardiovascular system suffer from it, the risk of a stroke increases. Since 2002 fermentozamestitelny therapy with use recombinant is available to treatment of illness of Fabri and-galaktotserebrozidazy.