Amino Acid Metabolism

Amino acid metabolism
Nitrogen balance
Dietary protein amino acid pool N excretion

(NH4+. urea)
protein catabolism,
synthesis biosynthesis
normal N balance: N ingested = N excreted

negative N balance: N ingested < N excreted
positive N balance: N ingested > N excreted
excess dietary amino acids (in excess over that required for
protein synthesis) are not stored but are degraded and
carbon skeletons used for glucose biosynthesis or energy
production.
proteins are constantly turning over and must therefore be

constantly replaced by protein synthesis. This requires a
steady supply of all 20 amino acids.
Protein sparing: if carbohydrate or fat intake is inadequate,

some dietary protein will be used for energy production,
reducing availability of amino acids for protein synthesis.
As carbohydrate and fats in diet increase, need for dietary
protein decreases.
Kwashiorkor: adequate caloric intake, but inadequate

protein intake.
Amino acid catabolism
accounts for ~ 10% of energy requirement of adults
When:
excess protein in diet
protein degradation exceeds demand for new protein
starvation when carbohydrates are not available
protein storing seeds such as beans, peas, etc.
Glucogenic vs ketogenic amino acids

ketogenic: yield AcCoA or AcAc as end products of
catabolism
glucogenic: are degraded to pyruvate or a member of
the TCA cycle (succinylCoA, OAA, -ketoglutarate,
fumarate). In absence of sugars, glucogenic amino
acids permit continued oxidation of fatty acids by
maintaining TCA cycle intermediates.
glucogenic and ketogenic: yield both ketogenic and
glucogenic products.
ile, phe, tyr and trp are glucogenic. leu and lys are
ketogenic. All others are glucogenic.
N catabolism
general strategy:

removal of N from amino acid by transamination
(generally first or second step of amino acid catabolic
pathways)

collection of N in glutamic acid

deamination of glutamic acid with release of NH4+

Removal of NH4+ by : i. secretion; or ii. conversion to
urea or other less toxic form.
i and ii. Transamination; see text p 537 and fig 17.7. see also
section 7.7, p212 on pyridoxal phosphate.
iii. glutamate dehydrogenase (see p 533 for reaction)

located in mitochondria
operates near equilibrium
iv. removal of NH4+

in liver by urea cycle and formation of urea
in other tissues collection of N in glutamine or alanine
for transport to liver
formation of glutamine -glutamine synthetase (see
fig 17.4)
glutamine transported to liver or kidney
where it is broken down to glutamate and
NH4+ by glutaminase.
+
in kidney NH4 is secreted in urine with an
anion such as -OH butyrate.
+
in liver NH4 is used to make urea.
formation of alanine - "alanine glucose cycle"
in skeletal muscle pyruvate acts as acceptor
in transaminase reaction. Ala is transported
to the liver where it undergoes
transamination to yield pyruvate that is used
for gluconeogenesis. The glucose is released
and can return to muscle where is
glycolytically degraded back to pyruvate.
N metabolism in kidney
+
glutamine converted to glutamate + NH4 by
glutaminase and NH4+ is secreted in urine
along with an anion.
during acidosis glutamine is shunted from
liver to kidney to conserve bicarbonate in
the liver (ie less NH4+ used for synthesis of
urea) and extra NH4+ production in kidney is
secreted with anions (eg ketone bodies) in
urine.
-ketoglutarate produced in kidney is used
for production of HCO3-, which is released
to blood (see p 563) and glucose.
Urea cycle
occurs in liver mito and cyto
urea secreted in urine - up to 30 g/day
source of N - glutamate dehydrogenase,
glutaminase

Reactions of:

carbamylphosphate synthase - mito

produces carbamyl phosphate from 2
ATP, CO2 and NH4+

committed step

activated by N'Ac glutamate

ornithine transcarbamylase - mito

tightly coupled to carbamylphosphate
synthase so that carbamyl phosphate is
rapidly added to ornithine to form
citrulline

arginosuccinate synthtase - cyto

aspartate transported into mito in
exchange for glu.

arginosuccinate lyase - cyto

yields arginine and fumarate.

fumarate used for synthesis of glucose
(fumarate malate OAA PEP
etc.

arginase - cyto

yields urea and ornithine

ornithine transported back into mito

absent from kidney which cannot make
urea but is a source of arginine.
Interorgan relationships in N metabolism
Epithelial cells
Several
of intestine
steps
GluNH2 cittruline
GluNH2
Liver
Kidney
cittruline
Arg Arginine Arginine

Urea
Urea Several
cycle steps
Ornithine
Several
steps creatine
glutamate
To urine
Muscle
creatine
P-creatine
creatinine non-enzymatic
Adapted from Devlin,

Biochemistry with Clinical Corrleation
4th ed.
Alanine - glucose cycle
Muscle
glucose
2 pyruvate
-aa
-ka 2 alanine
glucose 2 alanine
Liver
2 alanine
glucose
-ka
2 pyruvate -aa

Amino Acid Metabolism

Uploaded by

Copyright:

Available Formats

Amino Acid Metabolism

Uploaded by

Document Information

Original Description:

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Amino Acid Metabolism

Uploaded by

Copyright:

Available Formats

Amino acid metabolism

Dietary protein amino acid pool N excretion

normal N balance: N ingested = N excreted

proteins are constantly turning over and must therefore be

Protein sparing: if carbohydrate or fat intake is inadequate,

Kwashiorkor: adequate caloric intake, but inadequate

accounts for ~ 10% of energy requirement of adults

Glucogenic vs ketogenic amino acids

iii. glutamate dehydrogenase (see p 533 for reaction)

iv. removal of NH4+

Arg Arginine Arginine

Adapted from Devlin,

You might also like