AMINO ACIDS

METABOLISM
Digestion and Absorption of Proteins
 PROTEIN TURNOVER OCCURS
IN ALL FORMS OF LIFE

Each day humans turn over 1–2% of their total

body protein, principally muscle protein

Of the liberated amino acids, approximately 75%

are reutilized

Excess amino acids are rapidly degraded

Ubiquitin Tags Proteins for Destruction
ANIMALS CONVERT α-AMINO NITROGEN TO
VARIED END PRODUCTS

Ammonotelic : excrete ammonia (teleostean fish)

Uricotelic : excrete uric acid (birds)

Ureotelic : excrete urea (land animals including

human)
 BIOSYNTHESIS OF UREA

3 AMMONIA TRANSPORT

4
 TRASAMINATION

except lysine, threonine, proline, and hydroxyproline

 TRANSAMINASI

Alanine-pyruvate aminotransferase
(alanine aminotransferase)

glutamate-α-ketoglutarate aminotransferase
(glutamate aminotransferase)
OXIDATIVE DEAMINATION
OXIDATIVE DEAMINATION

L-glutamate
dehydrogenase
Glutamine Synthase Fixes Ammonia
as Glutamine
NH3 metabolism in the liver
UREA CYCLE
Metabolic Integration of Nitrogen Metabolism
 MITOCHONDRIA

CYTOSOL

UREA CYCLE
(LIVER)
Ammonia Intoxication Is Life-Threatening

Symptoms of ammonia intoxication

include tremor, slurred speech, blurred
vision, coma, and ultimately death.
METABOLIC DISORDERS ARE ASSOCIATED WITH
EACH REACTION OF THE UREA CYCLE
All defects in urea synthesis result in ammonia intoxication

Clinical symptoms of urea cycle disorders : include vomiting,

intermittent ataxia, irritability, lethargy, and mental retardation

Hyperammonemia Type 1. Carbamoyl phosphate synthase I deficiency

Hyperammonemia Type 2. A deficiency of ornithine transcarbamoylase

Citrullinemia. In this rare disorder, plasma and cerebrospinal fluid citrulline

levels are elevated and 1–2 g of citrulline are excreted daily.

Argininosuccinicaciduria. Absence of argininosuccinase

Hyperargininemia. Low erythrocyte levels of arginase

Catabolism of the Carbon Skeletons of Amino Acids
Fates of the Carbon Skeletons of Amino Acids
Amino acid biosynthesis: overview
NUTRITIONALLY NONESSENTIAL AMINO ACIDS HAVE
SHORT BIOSYNTHETIC PATHWAYS
Inborn errors of amino acid metabolism
DNA Replication
TRANSCRIPT
Structure of Eukaryotic Ribosomes
General Structure of tRNA Molecules
The genetic code (codon assignments in mammalian messenger RNA)
Features of the genetic code
Formation of aminoacyl-tRNA.
Recognition of the codon by the anticodon
 ATP + Pi

ATP
BINDING OF
AMINOACYL-TRNA
TO THE A SITE

PEPTIDE BOND
FORMATION
(peptidyltransferase) TRANSLOCATION
Mechanism of Protein Synthesis
Termination Process of Protein Synthesis
regulation on gene expression.
The mechanism of repression and derepression of the lac operon
Antibiotic inhibitors of protein synthesis