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LUDI Amino Acid Metabolism
n/a
61
Dentistry
Undergraduate 2
04/11/2014

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Term
What enzyme type breaks down proteins in the GI tract?
Definition
Proteolytic
Term
What do proteolytic enzymes break proteins down into?
Definition
Amino acids and oligopeptides
Term
What breaks down oligopeptides further?
Definition
Aminopeptidases
Term
What do aminopeptidases break oligopeptides into?
Definition
Di/Tri-peptides
Term
What now happens to Di/Tri-peptides
Definition
Broken down further by peptidases into amino acids
Term
Where does this occur?
Definition
Lumen of GI tract and intestinal cell
Term
What tags proteins for degredation?
Definition
Ubiquitin
Term
What happens to a ubiquitinated protein?
Definition
It is broken down by a proteasome into peptide fragments and ubiquitin
Term
What process turns peptide fragments into amino acids?
Definition
Proteolysis
Term
What are the fates of the amino acids from protein degredation? (3)
Definition
1. Left intact for biosynthesis
2. Carbon skeleton produced
3. Amine group removed
Term
Carbon skeleton uses (3)
Definition
1. Glucose/glycogen synthesis
2. Cellular respiration
3. FA synthesis
Term
What process removes the amine group from amino acids?
Definition
Oxidative degredation
Term
What is the initial acceptor for NH4?
Definition
Glutamate
Term
First stage in oxidative degredation?
Definition
Amine group is removed from the amino acid
Term
Which enzyme removes the amine group from the amino acid?
Definition
Transaminase
Term
What is the amine group transferred to?
Definition
Alpha-ketoglutarate
Term
What enzyme then acts on glutamate?
Definition
Glutamate dehydrogenase
Term
What is NAD+ required for?
Definition
Oxidising glutamate
Term
What happens after NH4 has been removed?
Definition
Alpha-ketoglutarate is reformed
Term
What is the fate of the released NH4?
Definition
The urea cycle
Term
What is pyroxidine commonly known as?
Definition
Vitamin B6
Term
What role does pyroxidine play in oxidative degredation?
Definition
It is a co-factor
Term
Why is the first step of the urea cycle called the committed step
Definition
It is where the urea cycle is regulated
Term
What is produced during the committed step?
Definition
Carbamoyl Phosphate
Term
What is used to produce carbamoyl phosphate?
Definition
Bicarbonate (from CO2) and NH3
Term
What provides the energy for the committed step?
Definition
Two ATP molecules
Term
Where does the committed step take place?
Definition
In the mitochondrial matrix
Term
What molecule positively regulates the committed step?
Definition
N-acetylglutamate
Term
What does carbamoyl phosphate combine with in the urea cycle?
Definition
Ornithine
Term
What does carbamoyl phosphate and ornithine produce?
Definition
Citrulline
Term
What does citrulline combine with?
Definition
Aspartate
Term
Which two molecules provide nitrogen atoms?
Definition
Carbamoyl phosphate and aspartate
Term
What provides energy for the addition of citrulline and aspartate?
Definition
ATP --> AMP + PPi
Term
What does citurlline and aspartate combining using ATP produce?
Definition
Argininosuccinate
Term
What does argininosuccincate give off?
Definition
Fumarate
Term
What is the destination of fumarate?
Definition
TCA cycle
Term
What is produced when argninosuccinate gives off fumarate?
Definition
Arginine
Term
What combines with arginine next?
Definition
H2O
Term
What is given off?
Definition
Urea
Term
Chemical formula for urea?
Definition
H2N-C=O-NH2
Term
How many nitrogen molecules in urea?
Definition
Two
Term
How can metabolic integration occur with the urea cycle?
Definition
Fumarate produced can enter the TCA cycle. Oxaloacetate can produce aspartate which enters the urea cycle
Term
Net formula for the urea cycle
Definition
CO2 + NH4 + 2H2O + Aspartate --> Fumarate + Urea
Term
Net energy formula for the urea cycle
Definition
3ATP --> 2ADP + AMP + PPi + Pi
Term
Cause of inborn errors of urea cycle
Definition
Genetic deficiency in each enzyme of the urea cycle
Term
Most common enzyme deficiency for the urea cycle
Definition
Ornithine transcabamoylase
Term
What do urea cycle enzyme deficiencies cause?
Definition
A failure to synthesis urea leading to ammonia toxicity
Term
What other disease can ammonia toxicity?
Definition
Cirrhosis
Term
How can urea cycle failure be treated?
Definition
Glycine and glutamine can be eliminated by simple pharamaceuticals
Term
Two types of amino acid skeletons
Definition
Glucogenic and ketogenic
Term
Fate of glucogenic amino acids
Definition
Gluconeogenesis/FA synthesis
Term
Fate of ketogenic amino acids
Definition
alternative fuel source during starvation
Term
What can some amino acids be?
Definition
Both glucogenic and ketogenic
Term
Which amino acid is purely ketogenic?
Definition
Lysine
Term
What type of amino acid is phenylalanine?
Definition
Ketogenic
Term
What is phenylalanaine eventually converted to?
Definition
Fumarate and Acetoacetate
Term
What is phenylalanine first converted to?
Definition
Tyrosine
Term
What enzyme converts phenylalanine to tyrosine?
Definition
A phenylalanine hydroxylase
Term
What is phenylketonuria?
Definition
A deficiency of phenylalanine hydroxylase
Term
What occurs during phenylketonuria? (4)
Definition
1. Phenylalanine not converted to tyrosine
2. Phenylpyruvate produces instead
3. Alpha-amino acid produced instead
4. Blood pH drops due to Alpha-amino acid
Term
Amino acids as precursors (4)
Definition
1. Nucleic acids
2. Hormones
3. Neurotransmitters
4. Co-factors
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