Metabolism, N-Skeleton, Transamination, Oxidative & De Amination By Dr Pooja For MBBS 1st Proff

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Metabolism, N-Skeleton, Transamination, Oxidative & De Amination -
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The metabolism of nitrogen-containing compounds involves the processing of amino acids to remove their amino groups. Key processes include transamination, oxidative deamination, and non-oxidative deamination. These pathways are crucial for the catabolism of amino acids and the synthesis of new nitrogen-containing compounds.

Transamination -
Definition -
The transfer of an amino group from an amino acid to an α-keto acid.

Enzymes -
Aminotransferases (transaminases), such as alanine aminotransferase (ALT) and aspartate aminotransferase (AST).

Coenzyme -
Pyridoxal phosphate (PLP), derived from vitamin B6.

Process -
An amino acid (e.g., alanine) donates its amino group to an α-keto acid (e.g., α-ketoglutarate), forming a new amino acid (e.g., glutamate) and a new α-keto acid (e.g., pyruvate).

Significance -
Facilitates the redistribution of amino groups.
Essential for the synthesis of non-essential amino acids.

Oxidative Deamination -

Definition -
The removal of an amino group from an amino acid as free ammonia, with the concurrent production of an α-keto acid.

Primary Enzyme -
Glutamate dehydrogenase (GDH).

Coenzymes -
NAD+ or NADP+.

Process -
Glutamate is deaminated to α-ketoglutarate and ammonia (NH3), catalyzed by GDH.
NAD+ or NADP+ is reduced to NADH or NADPH, respectively.

Location -
Mainly occurs in the liver and kidney mitochondria.

Significance -

Provides α-keto acids for energy metabolism or gluconeogenesis.
Supplies ammonia for the urea cycle.

Non-Oxidative Deamination -
Definition -

Removal of an amino group from amino acids without the involvement of oxygen.
Common Enzymes

Serine dehydratase (for serine).
Threonine dehydratase (for threonine).
Process

Serine is converted to pyruvate and ammonia by serine dehydratase.
Threonine is converted to α-ketobutyrate and ammonia by threonine dehydratase.
Significance

Direct removal of amino groups from certain amino acids.
Important for the catabolism of serine and threonine.

Clinical Relevance -

Hyperammonemia -
Elevated levels of ammonia in the blood due to defects in enzymes involved in these pathways.
Can lead to neurological impairments and require medical intervention.

Liver Diseases -
Liver dysfunction can impair these metabolic processes, leading to imbalances in amino acid and nitrogen metabolism.
Understanding these pathways is essential for diagnosing and managing metabolic disorders and for comprehending the overall nitrogen balance in the body. 🌟🧬🩺



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