How Do You Spell ARGININOSUCCINATE SYNTHETASE?

1. Argininosuccinate synthetase is an enzyme that plays a vital role in the urea cycle, a metabolic pathway that occurs primarily in the liver and helps detoxify ammonia, a harmful waste product of protein metabolism. The enzyme is encoded by the ASS1 gene and catalyzes the formation of argininosuccinate, an intermediate compound in the urea cycle.

   Specifically, argininosuccinate synthetase combines the amino acid citrulline and adenosine triphosphate (ATP) to form argininosuccinate. This reaction requires the presence of magnesium ions as a cofactor. Argininosuccinate can then be further processed and converted into arginine and fumarate in subsequent steps of the urea cycle.

   The term "synthetase" in the enzyme's name indicates its role in catalyzing a synthesis reaction, wherein two smaller molecules are joined together to create a larger compound. In this case, argininosuccinate synthetase is responsible for the synthesis of argininosuccinate from citrulline and ATP.

   Deficiencies or mutations in the ASS1 gene can result in the metabolic disorder known as citrullinemia, where the enzyme activity is impaired. This can lead to the accumulation of toxic levels of ammonia in the blood, causing symptoms such as vomiting, lethargy, and neurological problems. Treatment options for citrullinemia often include dietary modifications and medications to help manage ammonia levels in the body.

Common Misspellings for ARGININOSUCCINATE SYNTHETASE

The word "argininosuccinate synthetase" has its etymology rooted in the individual components of the term.

1. Arginino-: This part of the word comes from "arginine", which is an amino acid that is derived from the Latin word "argūrus", meaning "shiny" or "silver". The term "arginine" itself was coined by the German chemist Ernst Schulze in 1886.

2. -succinate: This part of the word is derived from "succinic acid", which is a dicarboxylic acid. The term "succinic" comes from the Latin word "succinum", which means "amber". This is because succinic acid was first extracted from amber resin, which is fossilized tree resin.

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