Podcast
Questions and Answers
Which of the following is a nonprotein nitrogen-containing compound synthesized from amino acids?
Which of the following is a nonprotein nitrogen-containing compound synthesized from amino acids?
- Porphyrins (correct)
- Fatty acids
- Calcium
- Glucose
What is the primary role of the hepatic enzyme phenylalanine hydroxylase (PAH)?
What is the primary role of the hepatic enzyme phenylalanine hydroxylase (PAH)?
- Regulation of blood glucose levels
- Breakdown of fatty acids
- Production of histamine
- Metabolism of phenylalanine to tyrosine (correct)
In which organ is ammonium produced during deamination of amino acids converted into urea?
In which organ is ammonium produced during deamination of amino acids converted into urea?
- Kidneys
- Spleen
- Pancreas
- Liver (correct)
Which of the following compounds is necessary to metabolize the amino acid phenylalanine to tyrosine?
Which of the following compounds is necessary to metabolize the amino acid phenylalanine to tyrosine?
Which genetic disorder is characterized by a deficiency in the hepatic enzyme phenylalanine hydroxylase (PAH)?
Which genetic disorder is characterized by a deficiency in the hepatic enzyme phenylalanine hydroxylase (PAH)?
What percentage of body energy is due to proteins?
What percentage of body energy is due to proteins?
What is the primary cause of the musty odor in the urine of individuals with PKU?
What is the primary cause of the musty odor in the urine of individuals with PKU?
In which variant of PKU are phenylalanine levels typically between 600 and 1200 μmol/L?
In which variant of PKU are phenylalanine levels typically between 600 and 1200 μmol/L?
What is the rare form of PKU that occurs when there is a defect in the biosynthesis of the cofactor tetrahydrobiopterin (BH4)?
What is the rare form of PKU that occurs when there is a defect in the biosynthesis of the cofactor tetrahydrobiopterin (BH4)?
What damage can occur if PKU is left untreated?
What damage can occur if PKU is left untreated?
What type of assay is used for screening PKU in infants?
What type of assay is used for screening PKU in infants?
What is the metabolic antagonist to B. subtilis growth used in the Guthrie bacterial inhibition assay?
What is the metabolic antagonist to B. subtilis growth used in the Guthrie bacterial inhibition assay?
At what blood level does the Guthrie bacterial inhibition assay indicate that bacterial growth occurs?
At what blood level does the Guthrie bacterial inhibition assay indicate that bacterial growth occurs?
What is necessary to avoid false-negative results in the Guthrie bacterial inhibition assay?
What is necessary to avoid false-negative results in the Guthrie bacterial inhibition assay?
In which variant of PKU do phenylalanine levels range from 180-600 μmol/L with no accompanying accumulation of phenylketones?
In which variant of PKU do phenylalanine levels range from 180-600 μmol/L with no accompanying accumulation of phenylketones?
