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Amino Acid 1

Amino acid


Vignette: A 25-year-old man comes to the clinic complaining of chronic fatigue and muscle weakness. He mentions that he has a family history of a similar condition. He undergoes several tests, and results show elevated levels of a certain organic compound in his blood. Further examination reveals that he has a deficiency in the enzyme that removes the amine group from this compound, leading to its accumulation. Which of the following is the most likely organic compound that is accumulating in this patient's blood?


A. Glucose

B. Glycine

C. Glutamic acid

D. Alanine

E. Aspartic acid


C. Glutamic acid


The patient's symptoms and family history suggest a genetic metabolic disorder. The key to this question is recognizing that the process of removing an amine group from an organic compound is called deamination, which primarily occurs in the liver. Among the choices given, both glutamic acid and aspartic acid can undergo deamination, but glutamic acid is the principal amino acid involved in this process. In the body, glutamic acid can be deaminated to form alpha-ketoglutarate, a component of the citric acid cycle. If an individual has an enzyme deficiency that hinders this process, it could lead to an accumulation of glutamic acid in the blood, resulting in the patient's symptoms. Therefore, the correct answer is C. Glutamic acid.


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