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Questions and Answers
What is the characteristic of D-a.a. in proteins?
What is the characteristic of D-a.a. in proteins?
What determines the kind of amino acid?
What determines the kind of amino acid?
What is the classification of aspartate?
What is the classification of aspartate?
What is the characteristic of glycine?
What is the characteristic of glycine?
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What is the α–carbon in an amino acid?
What is the α–carbon in an amino acid?
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What is the function of biocatalysts in living cells?
What is the function of biocatalysts in living cells?
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What is the primary consequence of α–KG depletion in the TCA cycle?
What is the primary consequence of α–KG depletion in the TCA cycle?
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What is the consequence of a complete block in any of the steps of the urea cycle?
What is the consequence of a complete block in any of the steps of the urea cycle?
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What is the role of α-keto analogues in the treatment of hyperammonemia?
What is the role of α-keto analogues in the treatment of hyperammonemia?
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What is the outcome of a high concentration of NH4+ in the brain?
What is the outcome of a high concentration of NH4+ in the brain?
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What is the outcome of glutamate formation in the brain?
What is the outcome of glutamate formation in the brain?
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Which enzyme is responsible for funneling amino groups from a variety of compounds to Glutamate for conversion to Ammonia?
Which enzyme is responsible for funneling amino groups from a variety of compounds to Glutamate for conversion to Ammonia?
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What is the byproduct of the transamination reaction between Glutamate and α-Ketoglutarate?
What is the byproduct of the transamination reaction between Glutamate and α-Ketoglutarate?
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What is the characteristic of Portal System Encephalopathy (PSE)?
What is the characteristic of Portal System Encephalopathy (PSE)?
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Which two amino acids are the primary donors of amino groups to the urea cycle?
Which two amino acids are the primary donors of amino groups to the urea cycle?
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What is the role of Glutamate-Aspartate Aminotransferase in amino acid metabolism?
What is the role of Glutamate-Aspartate Aminotransferase in amino acid metabolism?
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What is the end product of the degradation of most amino acids in amino acid metabolism?
What is the end product of the degradation of most amino acids in amino acid metabolism?
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What is the enzyme that precedes the urea cycle in amino acid metabolism?
What is the enzyme that precedes the urea cycle in amino acid metabolism?
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What is the result of a defect in the enzyme phenylalanine hydroxylase?
What is the result of a defect in the enzyme phenylalanine hydroxylase?
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What is the byproduct of the reaction catalyzed by the enzyme homogentisate oxidase?
What is the byproduct of the reaction catalyzed by the enzyme homogentisate oxidase?
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What is the precursor molecule to melanin synthesis?
What is the precursor molecule to melanin synthesis?
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What is the result of a defect in the enzyme dihydropterin reductase?
What is the result of a defect in the enzyme dihydropterin reductase?
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What is the enzyme that catalyzes the conversion of phenylalanine to tyrosine?
What is the enzyme that catalyzes the conversion of phenylalanine to tyrosine?
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What is the result of a defect in the enzyme tyrosinase?
What is the result of a defect in the enzyme tyrosinase?
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Which amino acid is converted to oxAA through transamination with α-KG?
Which amino acid is converted to oxAA through transamination with α-KG?
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What is the biochemical defect in individuals with Maple Syrup Disease?
What is the biochemical defect in individuals with Maple Syrup Disease?
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Which of the following amino acids is not a member of the C5 Family?
Which of the following amino acids is not a member of the C5 Family?
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What is the result of the blockage of oxidative decarboxylation of branched-chain amino acids?
What is the result of the blockage of oxidative decarboxylation of branched-chain amino acids?
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What is the characteristic feature of the urine of individuals with Maple Syrup Disease?
What is the characteristic feature of the urine of individuals with Maple Syrup Disease?
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What is the consequence of the absence of α-keto acid decarboxylase enzyme in individuals with Maple Syrup Disease?
What is the consequence of the absence of α-keto acid decarboxylase enzyme in individuals with Maple Syrup Disease?
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Study Notes
Biopolymers and Monomer Units
- Biopolymers are constructed from distinct monomer units/building blocks.
- For nucleic acids, the monomer units are nucleotides.
- For proteins, the monomer units are L-α-amino acids.
Amino Acids
- α-amino acids have both an amino and a carboxylic acid function attached to the same carbon atom.
- There are different classes of amino acids, including:
- Diaminomonocarboxylic acid (positively charged, e.g., lysine)
- Monoaminodicarboxylic acid (negatively charged, e.g., aspartate)
- Polar or non-polar (hydrophilic/hydrophobic)
- Glycine, with R=H, has no optical activity.
- Although D-amino acids occur in cells and in polypeptides, they are not present in proteins.
Amino Acid Structure and Classification
- Amino acids have trivial names (e.g., glycine, tryptophan) and systematic chemical names.
- The carbon bearing the C=O and NH2 groups is the α–carbon, and the next is β-carbon.
Urea Cycle and Hyperammonaemia
- Inherited hyperammonaemia is caused by genetic defects of various enzymes of the urea cycle.
- High levels of NH3 are toxic to humans and can lead to coma or death.
- Treatment involves a low protein diet and α-keto analogues.
Transamination Reactions
- Transamination reactions involve the transfer of amino groups from a variety of compounds to glutamate for conversion to ammonia.
- Alanine transaminase and glutamate dehydrogenase funnel amino groups from various compounds to glutamate.
- These reactions are reversible.
Amino Acid Metabolism
- Amino acid metabolism involves the degradation of amino acids to glutamate or aspartate, which are interconverted by glutamate-aspartate aminotransferase.
- The amino group of most amino acids is funneled into the formation of glutamate or aspartate, which are then converted to ammonia.
Inborn Errors of Phenylalanine Metabolism
- Phenylketonuria is caused by a lack of phenylalanine hydroxylase, leading to the accumulation of phenylalanine.
- Tyrosinemia is caused by a defect of tyrosine transaminase, leading to the accumulation of tyrosine.
- Albinism is caused by a defect of tyrosinase, leading to the accumulation of tyrosine.
- Alcaptonuria is caused by a defect of homogentisate oxidase, leading to the accumulation of homogentisate.
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Description
Test your understanding of biopolymers, including proteins and nucleic acids, and their composition of distinct monomer units. Learn about the structure of these biomolecules and their role in living cells.