Metabolism of Individual Amino Acids — MCQs

Which amino acids accumulate in maple syrup urine disease?

Because free ammonia in the blood is toxic to the body, it is transported in which of the following non-toxic forms?

An infant presented with vomiting, malnutrition, blue eyes, blonde hair & fair skin. On investigation, Guthrie test was positive. All are true regarding this disease EXCEPT:

In a patient with maple syrup urine disease, all of the following amino acids should be restricted in diet except?

Which of the following is not a substrate for gluconeogenesis?

Which of the following compounds is not formed with the involvement of glycine?

Which dietary deficiency of a vitamin can cause pellagra?

Pyridoxine deficiency leads to altered metabolism of?

A 2-month-old infant presents with vomiting, lethargy, and metabolic acidosis. Blood tests reveal elevated levels of ammonia. What is the most likely diagnosis?

Methionine can enter the TCA cycle at which level?