Vous êtes sur la page 1sur 60

AMINO ACIDS

METABOLISM

Digestion and Absorption of Proteins

PROTEIN TURNOVER OCCURS


IN ALL FORMS OF LIFE
Each day humans turn over 12% 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 -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

AMMONIA TRANSPORT

TRASAMINATION

except lysine, threonine, proline, and hydroxyproline

TRANSAMINASI

Alanine-pyruvate aminotransferase
(alanine aminotransferase)

glutamate--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 12 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

Vous aimerez peut-être aussi