30 Questions
In which part of the cell is carbamoyl phosphate generated?
Mitochondria
What is the role of ornithine carbamoyl transferase?
To transfer the carbamoyl group to ornithine
What is the byproduct of the reaction catalyzed by carbamoyl phosphate synthetase I?
ATP
What is the end product of the reaction catalyzed by ornithine carbamoyl transferase?
Citrulline
Where does ornithine come from before entering the mitochondria?
Cytosol
What is the nature of the reaction catalyzed by carbamoyl phosphate synthetase I?
Irreversible
What is the result of the deficiency of homogentsatic oxygenase?
Alcaptonuria
What is the effect of lack of melanin in albinos?
Increased sensitivity to sunlight
What is the age group most affected by Parkinson's disease?
People over 60 years old
What is the result of the deficiency of p-OH phenyl pyruvate oxygenase/ hydroxylase?
Tyrosinaemia
What is the effect of the lack of pigment in the eye in albinos?
Photophobia
What is the result of the oxidation of homogentisate?
Formation of a dark pigment
What is the primary reason behind the abnormal development of the foetus in pregnant women with high phenylalanine levels?
Accumulation of phenylalanine
What effect can dietary control of phenylalanine levels have on preventing mental retardation?
Prevents mental retardation
In the context of phenylalanine accumulation, what might happen to the transport of other amino acids to the brain?
Inhibited transport
Which amino acid disorder is specifically linked with poor psychomotor maturation, hypopigmentation, and seizures?
Phenylketonuria
What is the key role of tyrosine transaminase deficiency in tyrosinaemia?
Causes excessive accumulation of tyrosine and its metabolites
What is the shared clinical manifestation between tyrosinaemia and phenylketonuria?
Hypopigmentation
What is the enzyme responsible for catalyzing the synthesis of citrulline in the mitochondria?
Citrulline synthetase
Which molecule provides the second amino group needed for the formation of urea?
Aspartate
What from ATP is cleaved during the condensation of aspartate and citrulline to form arginino succinate?
ATP
Which of the following is formed during the hydrolysis of PPi by pyrophosphatase?
Inorganic phosphate
What is the final product from the condensation of aspartate and citrulline?
Arginine
What is the driving factor for the condensation of aspartate and citrulline in the formation of arginino succinate?
ATP hydrolysis
Which enzyme is deficient in Maple Syrup Urine Disease?
α-keto acid dehydrogenase
What is the primary cause of brain dysfunction in Maple Syrup Disease?
High leucine levels
Which amino acids are affected in Maple Syrup Urine Disease?
Leucine, Isoleucine, Valine
What symptoms might be observed in an individual with Maple Syrup Disease?
Lethargy, Hypoglycemia, Convulsions
What is found in the urine of individuals with Maple Syrup Urine Disease that contributes to its distinct odor?
α-ketoisocaproic acid
Which enzyme is affected in Isovaleric Acidemia?
Isovaleryl-CoA dehydrogenase
Test your knowledge on the formation of carbamoyl phosphate in mitochondria, including the reaction process, enzymes involved, high energy compounds, and irreversibility. Find out how ATP, ADP, CO2, NH3, and phosphate synthetase I play a crucial role in this biochemical pathway.
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