metabolism of ammonia
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Metabolism of Ammonia Dr. Rohini C Sane
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Metabolism of AmmoniaNH3 → 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
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Functions of AmmoniaWaste 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 )
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PURINE BASE
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Disposal of Ammonia1) 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
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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 AmmoniaNH3 ↑
↓GLUTAMATE ↑
↓α KGA (intermediate of TCA CYCLE ) ↓
↓TCA ( impairment of TCA ) ↓
↓ATP in brain ↓
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Hyperammonemia
(a) Genetic defects in enzymes of Urea cycle (neonates )
↓
Mental retardation
(b) Acquired Hepatitis ( alcoholism- defective urea synthesis )
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Metabolic disorders of Urea cycleType of Hyperammonemmia Defective Enzyme
HYPER AMMONEMMIA I CPS I
HYPER AMMONEMMIA II Ornithine Trans carbomylase
HYPER AMMONEMMIA IIICitullinemia
Argino sucdefective encinatesynthtase
HYPER AMMONEMMIA IVArginosuccinic aciduria
Arginosuccinase
HYPER AMMONEMMIA VHyperargininemia
Arginase
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