Argininosuccinic acid
Argininosuccinic Acid
Argininosuccinic acid (pronunciation: ar-gi-no-suc-cin-ic acid) is a compound that plays a crucial role in the urea cycle, a series of biochemical reactions in mammals. During the urea cycle, nitrogen, a waste product of protein metabolism, is removed from the body.
Etymology
The term "Argininosuccinic acid" is derived from its chemical structure, which is a combination of arginine and succinic acid.
Biochemical Role
In the urea cycle, argininosuccinic acid is synthesized from citrulline and aspartate in a reaction catalyzed by the enzyme argininosuccinate synthase. This is then converted into arginine and fumarate by the enzyme argininosuccinate lyase.
Clinical Significance
A deficiency in argininosuccinate lyase can lead to a buildup of argininosuccinic acid in the body, causing a condition known as argininosuccinic aciduria. This is a rare genetic disorder that can cause a variety of symptoms, including intellectual disability, liver disease, and skin problems.
Related Terms
- Urea cycle
- Arginine
- Succinic acid
- Citrulline
- Aspartate
- Argininosuccinate synthase
- Argininosuccinate lyase
- Argininosuccinic aciduria
External links
- Medical encyclopedia article on Argininosuccinic acid
- Wikipedia's article - Argininosuccinic acid
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