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AMINOACID
METABOLISM
S.Malathi Varma
Assistant
Professor
GUTAMATE/GLUTAMINE- 50%
ESSENTIAL AMINOACIDS-10%
Protein
turnover
Transaminatio
n
FEATURES OF TRANSAMINATION
❑ Requires pyridoxal phosphate
❑ No free NH3, only transfer
❑ Reversible
❑ Responsible for syn.of non-essential aa
❑ It can divert excess aa towards energy generation
❑ The aminoacids undergo transamination to concentrate nitrogen into
glutamate
❑ All aa except lysine, threonine & proline/hydroxy proline undergo
transamination.
1. OXIDATIVE
2. NON-
OXIDATIVE
From transmination, the amino
groups generated are given to this.
Glutamate serves as collection
centre for NH2 groups
GDH
1. Zn –mitochondrial enzyme
2. Allosteric regulation
3. GTP,ATP inhibitors/GDP,ADP activators
4. Steroid/thyroid hormones inhibit
5. Protein rich meal,liver glutammate is elevated
FMN FMNH2
½
H20
catalase
Oxidative Deamination- By Oxidase
D-Aminoacids?
METABOLISM OF AMMONIA
1. Formation of NH4
4. Disposal of NH4
Transamination,deamination,purines,
pyrimidines,by action of bacterial urease
Ammoniotelic-aquatic animals
Uricotelic-reptiles/birds
Ureotelic-mammals
2,3,5-next page
2. TRANSPORT & STORAGE OF NH3:
• Conc of NH3- 10-20mg/dL(considerably low).
• NH3- immediately diverted to urea synthesis.
• Transport of NH3 between tissues & liver- Glutamine
or alanine.
• Glucose-alanine cycle
3.
Functions
of
NH3
Non-essential Aminoacids, Purines,
Pyrimidines
Amino sugars, Aspargine
Acid—Base balance
5. TOXICITY OF NH3: self study
Metabolism of Individual Amino-acids:
1. Glycine
2. Plenylalanine/Tyrosine
3. Leucine
4. Methionine
5. Serine
Ketogenic
L,K
Both
F,I,Y,W
Glycogenic
A,R,D,C,N,G,H,P,M,S,Th,V
Classification of Aminoacids based
On Fate of Carbon skeleton
MATTVILPH
Y
PHE- ESSENTIAL AMINOACID
TYR- NON-ESSENTIAL AMINOACID
Sparing action of tyrosine on phenylalanine
Degradation of tyrosine/phenylalanine
PLP-pyridoxal phosphate
-Melanin
/ Tyrosinase
SYNTHESIS OF THYROID HORMONES
Catecholamines
CATECHOL
SYNTHESIS OF CATECHOLAMINES
Functions of catecholamines
❑ Regulate carbohydrate & fat metabolism
❑ They stimulate breakdown of TAG/glycogen
❑ They cause increase in BP
❑ DOPA, Nor-Epinephrine- Neuro-transmitters in
BRAIN & Nervous system.
GLYCIN
E
GLYCIN
E
SERINE Serine hydroxy methyl transferase
THREONINE
aldolase
N5-N10THF
CO2, NH3 Glycine synthase
SYNTHESIS
DEGRADATION OF GLYCINE:
1. Oxidative deamination- NH3, CO2, N5-N10 THF.
2. Glycine can form Serine.
3. Synthesis of special products:
• Purine ring
• Glutathione
• Conjugation reactions
• Heme next page
• Creatine
Conjugation reactions
Detoxification – Benzoic acid Synthesis of Heme
(food preservative)
SYNTHESIS OF CREATINE
CREATININE
GLYCINE SYNTHETASE
SERINE HYDROXY METHYL TRANSFERASE
GLYCINE OXIDASE
TRANSAMINASE
PrimaryHyper oxaluria
- defect o glycine TA
❑ Increased urinry oxalate
❑ Deposition of oxalate in tissues
Disorders of GLYCINE
metabolism
Methionine- UTIIZATION, PRODUCTS
1
2
Condenses
with Serine
Enzyme
Cystathione
synthase
E-Methionine adenosyl transferase
E-Cystathionase
Homoserin
e
Note the role played by Vitamins
3
1
2
3
4
5
Leucine- Essential aa
• Genetic disease caused by deficiency of branched
-chain aminotransferase
• Alpha keto amino acids, valine, isolucine and
Lucine will accumulated in the blood and excreted
in the urine (smells like maple syrup)
• If untreated, mental retardation and early death
Maple syrup urine disease
Arginine- Essential aa
1
2
3
❑ The urea cycle is a cycle of biochemical reactions that produces urea
(NH2)2CO from ammonia (NH3). Urea accounts 80-90% Nitrogen.
❑ Animals that use this cycle, mainly amphibians and mammals, are
called ureotelic.
❑ The urea cycle converts highly toxic ammonia to urea for excretion.
❑ This cycle was the first metabolic cycle to be discovered (Hans Krebs
and Kurt Henseleit, 1932), five years before the discovery of the TCA
cycle.
❑ The urea synthesized in the liver and transportd to the kidneys for
excretion in the urine.
Urea Cycle
Easily soluble
Hypoalbuminemia-
Calcium
Tissue edema caused by-malnutrition(albumin syn )
Nephrotic syndrome( albumin lost through urine)
Liver cirrhosis( alb syn )
Chronic cardiac failure( hydrostatic pressure)

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Amino acid Metabolism - Nutritional Biochemistry