Metabolism of Phenylalanine and Tyrosine : Phenylketonuria (PKU), Alkaptonuria and Tyrosinemia
Dr.G Bhanu Prakash Animated Medical Videos・2 minutes read
Amino acids play essential roles in the body, with variations in structure and metabolism categories like glucogenic or ketogenic. Key amino acids like leucine and phenylalanine are crucial for various processes, including the formation of vital hormones and enzymes, but inborn errors related to these amino acids can lead to serious conditions.
Insights
- Amino acids have a common structure but differ in their R groups, impacting their metabolic pathways and categorization as glucogenic, ketogenic, or both.
- Tyrosine plays a crucial role in synthesizing various important compounds like melanin, thyroid hormones, and catecholamines, with its metabolism involving enzymatic reactions that can lead to significant health conditions if disrupted.
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Recent questions
What are the different categories of amino acids?
Glucogenic, ketogenic, or both.
What is the significance of tyrosine in the body?
Essential for melanin, thyroid hormones, and catecholamines synthesis.
How is tyrosine converted to epinephrine in the body?
Through a series of reactions involving enzymes and cofactors.
What are some inborn errors of metabolism related to amino acids?
Phenylketonuria, albinism, and tyrosinemia.
What is the role of leucine in amino acid metabolism?
Most ketogenic amino acid.
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Summary
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Amino Acid Metabolism and Inherited Disorders
- Amino acids have a common structure with variations in the R group.
- Amino acid metabolism is studied under two subheadings: nitrogen skeleton and carbon skeleton.
- Amino acids can undergo transamination leading to the formation of urea.
- Amino acids are categorized as glucogenic, ketogenic, or both.
- Leucine is the most ketogenic amino acid.
- Phenylalanine is an essential amino acid required for tyrosine synthesis.
- Tyrosine is crucial for the synthesis of melanin, t3 and t4 thyroid hormones, and catecholamines.
- Tyrosine is converted to epinephrine through a series of reactions involving enzymes and cofactors.
- Tyrosine catabolism involves several enzymatic steps leading to the formation of fumarate and acetoacetate.
- Inborn errors of metabolism related to phenylalanine and tyrosine can lead to conditions like phenylketonuria, albinism, and tyrosinemia.
