Argininosuccinate lyase deficiency is a
deficiency of the urea cycle. A result of this metabolic disorder is hyperammonemia,
or elevated ammonia levels.
Ammonia is a highly toxic biochemical by-product
which is the result of the metabolism of certain amino acids. In a person with
ASL Deficiency, the body is incapable of completing the process of converting
ammonia into urea, a less toxic form which can be easily removed from the body
primarily through urine.
Ammonia is harmful enough when it is
encountered in the environment, capable of causing burns on exposed surfaces.
On the inside of the body, it is capable of causing even more problems. Elevated
ammonia levels in the blood acts as a toxin and may affect the brain, resulting
in a number of symptoms, some of which include confusion, lethargy, and change
in mood, personality and behaviour among others, all of which are symptoms of
Argininosuccinate Lyase Deficiency.
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