Biochem 4 (Urea Cycle)

Proteins

Large molecules made up of amino acids that are essential for various biological functions.

Proteases

Enzymes that break down proteins by hydrolyzing peptide bonds.

Transamination

A biochemical process involving the transfer of an amino group from an amino acid to a keto acid.

Glutamate dehydrogenase

An enzyme involved in the oxidative deamination of glutamate in the liver.

Aminotransferases

Enzymes that catalyze transamination reactions; key examples include Alanine Aminotransferase (ALT) and Aspartate Aminotransferase (AST).

ALT (Alanine Aminotransferase)

An enzyme that transfers an amino group from alanine to α-ketoglutarate, forming pyruvate and glutamate.

AST (Aspartate Aminotransferase)

An enzyme that transfers an amino group from aspartate to α-ketoglutarate, producing oxaloacetate and glutamate.

Transamination Disorders

Metabolic disorders arising from defects in transamination enzymes, indicated by elevated ALT and AST levels.

Vitamin B6 Deficiency

A condition that impairs transamination reactions, leading to symptoms such as neurological disorders and anemia.

Deamination

The removal of an amino group from an amino acid or its derivative, crucial for amino acid breakdown.

Urea Cycle

A metabolic pathway that converts ammonia from amino acid deamination into urea for excretion.

Phenylketonuria (PKU)

A genetic disorder that prevents proper metabolism of phenylalanine, leading to its toxic accumulation.

Symptoms of PKU

Include intellectual disability, seizures, behavioral problems, microcephaly, eczema, and a musty odor in urine.

Treatment for PKU

Involves dietary management, low-phenylalanine diet, medical foods, and amino acid supplements.

Oxidative deamination

The process of removing amino groups from amino acids, primarily occurring in the liver.