20. 1.6 Cadena de electrones 1.Metabolismo de los carbohidratos
21.
22. Los Electrones (e-) viajan por los complejos. Los Protones (H+) viajan atreves de los complejos pasando de la matriz mitocondrial al espacio intermembranal. Provienen del NADH y FADH2 formado en CAT.
37. PYRUVATE DEHYDROGENASE COMPLEX DEFICIENCY Most children with this enzyme deficiency present in infancy with delayed development and reduced muscle tone often associated with ataxia and seizures. Some infants have congenital malformations of the brain. ¿why? Without mitochondrial oxidation, pyruvate is reduced to lactate. The ATP yield from anaerobic glycolysis is less than a tenth of that produced from complete oxidation of glucose via the tricarboxylic acid cycle. The diagnosis is suggested by elevated lactate, but with a normal lactate/pyruvate ratio, i.e. no evidence of hypoxia. A ketogenic diet and severe restriction of protein (<15%) and carbohydrate (<5%) improves mental development. Such treatment ensures that the cells use acetyl-CoA from fat metabolism. A few children show a reduction in plasma lactate on treatment with large doses of thiamine, but the outlook is generally poor.
38. El beri-beri se da por la deficiencia de tiamina, ¿Qué metabolitos medirías para saber si un paciente lo tiene?