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	<meta name="DC.Description" xml:lang="es" content=" La hiperamoniemia es un trastorno metabólico causado por la acumulación   de ion amonio en el organismo, produciendo alteraciones en el SNC. Este ion, sumamente tóxico, pierde su toxicidad al convertirse en urea mediante el proceso conocido como ciclo de la urea. Cuando se producen defectos en las enzimas y en el trasporte intermediario de los metabolitos implicados en este ciclo, se producen varios tipos de hiperamoniemia primaria que son en los que nos vamos a centrar. Niveles elevados de amoniaco pueden tener graves consecuencias para el SNC e incluso la muerte. Esto se debe a que la detoxificación de este compuesto requiere de alfa-ketoglutarato, glutamato y glutamina. En la hiperamoniemia se altera el equilibrio de la reacción de detoxificación, disminuyendo el alfa-cetoglutarato y el glutamato, y aumentando la glutamina. Al ser el alfa- cetoglutarato un intermediario del ciclo de Krebs se verán alterados los niveles de ATP. Además el glutamato es el principal neurotransmisor excitatorio del SNC, por lo que se verán afectados los circuitos nerviosos en los que este interviene. Por último, el aumento de glutamina, producirá un incremento del volumen de los astrocitos, células en las que tiene lugar su síntesis. "/>
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								<meta name="DC.Subject" xml:lang="es" content="Ciclo de la urea"/>
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	<div id="articleTitle"><h3>Hiperamoniemia</h3></div>
	<div id="authorString"><em>Marta Murillo Hernández, Sara Fernández Rodríguez</em></div>
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		<h4>Resumen</h4>
		<br />
		<div>La hiperamoniemia es un trastorno metabólico causado por la acumulación<strong> </strong>de ion amonio en el organismo, produciendo alteraciones en el SNC. Este ion, sumamente tóxico, pierde su toxicidad al convertirse en urea mediante el proceso conocido como ciclo de la urea. Cuando se producen defectos en las enzimas y en el trasporte intermediario de los metabolitos implicados en este ciclo, se producen varios tipos de hiperamoniemia primaria que son en los que nos vamos a centrar. Niveles elevados de amoniaco pueden tener graves consecuencias para el SNC e incluso la muerte. Esto se debe a que la detoxificación de este compuesto requiere de alfa-ketoglutarato, glutamato y glutamina. En la hiperamoniemia se altera el equilibrio de la reacción de detoxificación, disminuyendo el alfa-cetoglutarato y el glutamato, y aumentando la glutamina. Al ser el alfa- cetoglutarato un intermediario del ciclo de Krebs se verán alterados los niveles de ATP. Además el glutamato es el principal neurotransmisor excitatorio del SNC, por lo que se verán afectados los circuitos nerviosos en los que este interviene. Por último, el aumento de glutamina, producirá un incremento del volumen de los astrocitos, células en las que tiene lugar su síntesis.</div>
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		<h4>Palabras clave</h4>
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		<div>Hiperamoniemia; Ciclo de la urea; Amoniaco; Glutamato;</div>
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