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Questions and Answers
What is the coenzyme form of pyridoxine required for the enzyme's activity?
What is the coenzyme form of pyridoxine required for the enzyme's activity?
- Amino acid
- Vitamin B6
- Pyridoxal phosphate (correct)
- Pyridoxine
What is the function of pyridoxal phosphate in aminotransferases?
What is the function of pyridoxal phosphate in aminotransferases?
- Final transporter of amino groups
- Initial donor of amino groups
- Intermediate carrier of amino groups (correct)
- Terminal acceptor of amino groups
What type of linkage is formed between pyridoxal phosphate and the ε-amino group of a Lys residue?
What type of linkage is formed between pyridoxal phosphate and the ε-amino group of a Lys residue?
- Aldimine (Schiff base) linkage (correct)
- Peptide bond
- Hydrogen bond
- Disulfide bond
What is the form in which amino groups are transported in blood to the liver?
What is the form in which amino groups are transported in blood to the liver?
Where are the amino groups of amino acids from dietary proteins and cellular proteins incorporated into?
Where are the amino groups of amino acids from dietary proteins and cellular proteins incorporated into?
What is the result of the formation of a Schiff base between pyridoxal phosphate and the amino group of the amino acid?
What is the result of the formation of a Schiff base between pyridoxal phosphate and the amino group of the amino acid?
What is the product of the transamination reaction between glutamate and pyruvate in muscle?
What is the product of the transamination reaction between glutamate and pyruvate in muscle?
What is the purpose of the Glucose-Alanine Cycle?
What is the purpose of the Glucose-Alanine Cycle?
What is the enzyme involved in the transamination reaction between alanine and α-ketoglutarate in the liver?
What is the enzyme involved in the transamination reaction between alanine and α-ketoglutarate in the liver?
What is the fate of pyruvate produced in the liver through gluconeogenesis?
What is the fate of pyruvate produced in the liver through gluconeogenesis?
What is the purpose of glutamine synthetase in extrahepatic tissues?
What is the purpose of glutamine synthetase in extrahepatic tissues?
What is the byproduct of the deamination of glutamate in the liver?
What is the byproduct of the deamination of glutamate in the liver?
What is the term used to describe the connection between the urea cycle and the citric acid cycle?
What is the term used to describe the connection between the urea cycle and the citric acid cycle?
Where are the enzymes fumarase and malate dehydrogenase also present as isozymes?
Where are the enzymes fumarase and malate dehydrogenase also present as isozymes?
What is the product of the hydration of fumarate in the cytosol?
What is the product of the hydration of fumarate in the cytosol?
What is one possible fate of oxaloacetate?
What is one possible fate of oxaloacetate?
At what two levels can the activity of the urea cycle be regulated?
At what two levels can the activity of the urea cycle be regulated?
What enzyme's rate of synthesis can control the activity of the urea cycle?
What enzyme's rate of synthesis can control the activity of the urea cycle?
What is the purpose of supplementing the diet with benzoate and phenylacetate in Carbamoyl phosphate synthetase I and ornithine transcarbamoylase deficiency?
What is the purpose of supplementing the diet with benzoate and phenylacetate in Carbamoyl phosphate synthetase I and ornithine transcarbamoylase deficiency?
Why is a protein-free diet not a treatment option for deficient enzymes?
Why is a protein-free diet not a treatment option for deficient enzymes?
What is the difference between essential and non-essential amino acids?
What is the difference between essential and non-essential amino acids?
What is a symptom of ammonia intoxication?
What is a symptom of ammonia intoxication?
What is a possible reason for the toxic effect of ammonia?
What is a possible reason for the toxic effect of ammonia?
What is a common symptom of hyperammonemia?
What is a common symptom of hyperammonemia?
What is the product of the cleavage of the aromatic ring of homogentisate?
What is the product of the cleavage of the aromatic ring of homogentisate?
What is the name of the disease caused by a deficiency of phenylalanine hydroxylase or its tetrahydrobiopterin cofactor?
What is the name of the disease caused by a deficiency of phenylalanine hydroxylase or its tetrahydrobiopterin cofactor?
What is the end product of the reaction catalyzed by maleylacetoacetate isomerase?
What is the end product of the reaction catalyzed by maleylacetoacetate isomerase?
Which enzyme is responsible for the hydrolysis of 4-fumarylacetoacetate to fumarate and acetoacetate?
Which enzyme is responsible for the hydrolysis of 4-fumarylacetoacetate to fumarate and acetoacetate?
What is the characteristic of the urine in individuals with phenylketonuria?
What is the characteristic of the urine in individuals with phenylketonuria?
What is the alternative pathway of phenylalanine metabolism in individuals with phenylketonuria?
What is the alternative pathway of phenylalanine metabolism in individuals with phenylketonuria?
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