Galactose-1-phosphate

Galactose-1-phosphate is a sugar derivative which plays a key role in the metabolism of the monosaccharide galactose. It is produced from galactose by the enzyme galactokinase and is a critical intermediate in the Leloir pathway, the primary pathway of galactose metabolism.

Structure and Properties

Galactose-1-phosphate has the chemical formula C6H13O9P. It is a phosphate ester of galactose, with the phosphate group attached to the first carbon atom of the sugar molecule. This gives it a negative charge, which is important for its role in metabolic reactions.

Role in Metabolism

In the Leloir pathway, galactose-1-phosphate is converted to UDP-galactose by the enzyme galactose-1-phosphate uridylyltransferase. This reaction also involves uridine triphosphate (UTP), which donates a uridine diphosphate (UDP) group to the sugar molecule. The resulting UDP-galactose can then be used in the synthesis of glycoproteins and glycolipids.

Clinical Significance

Deficiencies in the enzymes that metabolize galactose-1-phosphate can lead to galactosemia, a serious metabolic disorder. This condition is characterized by an inability to properly metabolize galactose, leading to its accumulation in the body. Symptoms can include jaundice, hepatomegaly, cataracts, and developmental delay. Treatment typically involves a diet that is low in galactose.

See Also

• Galactose
• Galactokinase
• Galactose-1-phosphate uridylyltransferase
• Leloir pathway
• Galactosemia

Galactose-1-phosphate Resources
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