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Aspartylglycosaminuria

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Aspartylglycosaminuria (AGU) is the commonest disorder in glycoprotein degradation. The mode of inheritance is autosomal recessive.

The cause of AGU is a mutation in the gene for the enzyme glycosylasparaginase which results in the accumulation of various glycoasparagines in the tissues and body fluids..

Affected individuals show normal early development followed by a gradual decline begins in childhood. Young adults suffer severe mental and motor impairment and death.

There is no specific treatment.


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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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