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Biochemistry
Amino acid metabolism
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Created by
Terry Ross
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Cards (24)
What is the main focus of amino acid metabolism?
Degradation
of amino acids from proteins
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Why are amino acids risky to accumulate in the body?
They have key functions and regulate
metabolism
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Where does excess amino acid degradation primarily occur?
Liver
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What is protein turnover?
Regulated
degradation
and
synthesis
of proteins
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What happens to damaged proteins in the body?
They are
removed
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How is ammonia formed in the body?
Through
amino acid
breakdown
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Why is ammonium ion (NH4+) considered toxic?
It leads to severe
neurological
problems
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What is the major nitrogen-containing excretory molecule?
Urea
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What percentage of nitrogen excretion is in the form of urea?
80%
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What are the three steps in the synthesis of urea?
Transamination
,
deamination
,
urea cycle
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What role do aminotransferases play in amino acid metabolism?
They move
amino groups
between amino acids
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What is the function of glutamine synthase?
Adds
NH4+
to
glutamate
to form glutamine
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What are the major carriers of nitrogen in the blood to the liver?
Alanine
and
glutamine
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Where does deamination occur?
In the
liver
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What is generated as an end-product of the urea cycle?
Fumarate
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How is fumarate converted for transport back to the mitochondrial matrix?
Converted to malate via
malate-aspartate shuttle
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What happens to carbon skeletons after amino acid degradation?
Converted into major
metabolic
intermediates
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What are ketogenic amino acids degraded to?
Acetyl-CoA
or
acetoacetyl-CoA
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What can glucogenic amino acids be converted into?
Pyruvate
or TCA cycle intermediates
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What are the inherited disorders of amino acid degradation?
Alcaptonuria
: Blocked degradation of
phenylalanine
and
tyrosine
Maple syrup urine disease
: Blocked degradation of
valine
,
isoleucine
, and leucine
Phenylketonuria
: Accumulation of phenylalanine leads to
mental retardation
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What is the consequence of a defect in a urea cycle enzyme?
Accumulation of urea cycle
intermediates
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What happens to glutamine levels in the circulation with urea cycle disorders?
Glutamine
levels
increase
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What happens to α-ketoglutarate levels in urea cycle disorders?
They become too low to fix
ammonium
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What is the effect of elevated ammonia levels in the blood?
They are toxic for the
nervous system
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