METABOLISM

OF
AMINO ACIDS
Yulia Suciati

OVERVIEW OF AMINO ACID METABOLISM

ENVIRONMENT

ORGANISM

Biosynthesis

Ingested
protein

Protein
2

a
AMINO
ACIDS
c

Degradation
(required)

b
c
Purines
Pyrimidines
Porphyrins

Carbon
skeletons

Nitrogen
(ketogenic)
Urea
acetoacetate
acetyl CoA

(glucogenic)
Used for
energy

pyruvate
-ketoglutarate
succinyl-CoA
fumarate
oxaloacetate

NITROGEN BALANCE
Nitrogen balance = nitrogen ingested - nitrogen excreted
(primarily as protein) (primarily as urea)
Nitrogen balance = 0 (nitrogen equilibrium)
protein synthesis = protein degradation
Positive nitrogen balance
protein synthesis > protein degradation
Negative nitrogen balance
protein synthesis < protein degradation

N balance = Nin - Nout

Positive Nitrogen Balance

Negative Nitrogen Balance

1. Stress

2. Decreased Intake

3. Lack of an essential AA

Metabolic Pool of Amino Acids

Amino Acid Requirements of Humans

-------------------------------------------------------------------Nutritionally Essential Nutritionally Nonessential
-------------------------------------------------------------------Argininea
Alanine
Histidine
Asparagine
Isoleucine
Aspartate
Leucine
Cysteine
Lysine
Glutamate
Methionine
Glutamine
Phenylalanine
Glycine
Threonine
Proline
Tryptophan
Serine
Valine
Tyrosine
--------------------------------------------------------------------a
Nutritionally semiessential. Synthesized at rates
inadequate to support growth of children.

Glucose-alanine cycle
Alanine plays a special role in
transporting amino groups to liver.
Ala is the carrier of ammonia and of the carbon
skeleton of pyruvate from muscle to liver.
The ammonia is excreted and the pyruvate is
used to produce glucose, which is returned to
the muscle.

According to D. L. Nelson, M. M. Cox :LEHNINGER. PRINCIPLES OF BIOCHEMISTRY Fifth edition

The fate of the amino group during amino acid catabolism

TRANSAMINATION

Clinicaly important transaminases

Alanine--ketoglutarate transferase ALT
(also called glutamate-pyruvate transaminase GPT)
Aspartate--ketoglutarate transferase AST
(also called glutamate-oxalacetate transferase GOT)
Important in the diagnosis of heart and liver damage caused by heart
attack, drug toxicity, or infection.

ALT

UREA CYCLE

mitochondria
cytosol

Function: detoxification of ammonia

(prevents hyperammonemia)

FATE OF THE CARBON SKELETONS

Carbon skeletons are used for energy.

Glucogenic: TCA cycle intermediates

or pyruvate (gluconeogensis)
Ketogenic: acetyl CoA, acetoacetyl CoA,
or acetoacetate

Metabolism of some
selected amino acids

Histidine Metabolism:
Histamine Formation
H
N

+
NH3
CH2 CHCO 2 -

Histidine
decarboxylase

H
N

CH2 CH2NH2

Histidine

CO2

Histamine

Histamine:
Synthesized in and released by mast cells
Mediator of allergic response: vasodilation,
bronchoconstriction

Glycine oxidation to CO2

Glycine produced from serine or from the diet can also be oxidized by glycine
decarboxylase (also referred to as the glycine cleavage complex, GCC) to yield a
second equivalent of N5,N10-methylene-tetrahydrofolate as well as ammonia and
CO2.
Copy from: http://themedicalbiochemistrypage.org/amino-acid-metabolism.html

Aromatic
Amino
Acids

Tyrosine

Phenylalanine

Aceto
acetate

fumarate

glucose

ketone

Tryptophane

Dopa &
Dopamine

Melanine

Skatol &
Indol

Melatonin

Nor
epinephrin
&
epinephrine

Thyroxin

Anthranilic

Serotonin

Alanine

Acetoacetyl
CoA

Nicotinamide

Biosynthesis of Tyrosine from Phenylalanine

Phenylalanine hydroxylase is a mixed-function oxygenase: one atom of oxygen is

incorporated into water and the other into the hydroxyl of tyrosine. The reductant is the
tetrahydrofolate-related cofactor tetrahydrobiopterin, which is maintained in the reduced
state by the NADH-dependent enzyme dihydropteridine reductase

Phenylketonuria
Hyperphenylalaninemia - complete deficiency of phenylalanine
hydroxylase (plasma level of Phe raises from normal 0.5 to 2 mg/dL to
more than 20 mg/dL).
The mental retardation is caused by the accumulation of
phenylalanine, which becomes a major donor of amino groups in
aminotransferase activity and depletes neural tissue of ketoglutarate.
Absence of -ketoglutarate in the brain shuts down the TCA cycle and
the associated production of aerobic energy, which is essential to
normal brain development.
Newborns are routinelly tested for blood concentration of Phe.
The diet with low-phenylalanine diet.

ALHAMDULILLAH
SEMOGA BERMANFAAT
YS/2013