Metabolic Routes

Innate errors cause deficiency of degradation, synthesis, storage and even though protein transport, leading to the accumulation of substances, allowing defects in the end items and intermediate of metabolic routes (HUNSY; CALDATO, 2006; SIMMONS; SNUSTAD, 2008; TONIN, 2010). They are known currently about 500 types of innate errors of the metabolism, in a poporo of 1:1000 cases in individuals born with life (HUSNY; CALDATO, 2006; SIEM, 2011). As example of these innate errors of the metabolism (EIM), the illnesses of glycogen storage can be cited (GSD). Are originated fisiopatologias of related enzymatic defects with the accumulation of molecules of glycogen in fabrics. Table 2 describes seven main described types of the fourteen GSDs in literature (EMEDICINE, 2011).

Illnesses of storage of Deficient TipoEnzima Glycogen Iglicose-6-fosfatase Implications illness of Von Gierke acid IImaltase illness of Pompe IIIdesramificadoradoena de Cori IV transglucosidase illness of Andersen V miofosforilase illness of McArdle VIfosforilase hepticadoena of Hers VIIfosfofrutoquinasedoena Tauri Table 2: Some types of illnesses of storage of Suitable glycogen: 2 – OBJECTIVE To revise existing bibliography concerning the illnesses caused for the innate errors of the metabolism of frutose, and its implications. 3 – DEVELOPMENT 3,1 By glicoltica the glucose is one of energy molecules of great importance and use for the good physiological functioning of the human organism. degradation of this molecule unchains in a great production of energy under the Adenosina form Tri Fosfato (ATP), in the end of its oxidativo process. The metabolism of glucose molecules can give through two ways, being they aerbica and anaerbica. The first way if distinguishes from second for the fact of if getting a number of 38 energy molecules of ATP, while the second way if gets only 04 molecules. Although the anaerbica way to produce only 02 molecules of ATP in liquid balance, it is of real importance so that all the oxidativo process happens (ALBERTS et al., 2006; OAK; PIMENTEL, 2007).

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