2. Metabolism of Ammonia
NH3 → NH4 ⁺ ion ( exist ammonium ion at p H 7.4)
I .Formation of Ammonia :
a ) Transamination & Deamination of biogenic amines ,NH2 group of
Purines & Pyrimidine
b) Urea bacterial urease NH3
II . Transport & storage :
Conc of serum ammonia 10 -20 micro gram /dl
Efficient transport &immediate utilization of ammonia for urea
synthesis
Ammonia is transported as Glutamine & Alanine (Glucose –Alanine cycle
No free Ammonia in serum under physiological conditions
3.
4.
5. Functions of Ammonia
Waste products of nitrogen
Synthesis of compounds like
a) Non essential amino acids
b) Purines
c) Pyrimidine
d) Amino sugars
e) Asparagine
f) NH₄⁺(acid base balance )
7. Disposal of Ammonia
1) Ammonitelic : ammonia excreted in water eg aquatic animals
2) Uricotelic : ammonia excreted as uric acid eg reptiles & birds
3) Ureotelic : ammonia excreted as urea (non toxic ,water soluble )eg mammals
AMMONIA URIC ACID
UREA
8. Toxicity of Ammonia
Symptoms of Toxicity of Ammonia
• Marginal elevation –toxic to brain
• Slurring of speech
• Blurring of vision
• Tremors
• Coma
• Death
Biochemistry of Toxicity of Ammonia
NH3 ↑
↓
GLUTAMATE ↑
↓
α KGA (intermediate of TCA CYCLE ) ↓
↓
TCA ( impairment of TCA ) ↓
↓
ATP in brain ↓
10. Metabolic disorders of Urea cycle
Type of Hyperammonemmia Defective Enzyme
HYPER AMMONEMMIA I CPS I
HYPER AMMONEMMIA II Ornithine Trans carbomylase
HYPER AMMONEMMIA III
Citullinemia
Argino sucdefective encinate
synthtase
HYPER AMMONEMMIA IV
Arginosuccinic aciduria
Arginosuccinase
HYPER AMMONEMMIA V
Hyperargininemia
Arginase