Galaktóz1foszfátá

Galaktóz1foszfátá, also known as galactose-1-phosphate, is a crucial intermediate molecule in the metabolism of galactose. It is formed from galactose by the enzyme galactokinase, which phosphorylates galactose at the C1 position, using ATP as the phosphate donor. This reaction is the first step in the Leloir pathway, the primary route for galactose catabolism in most organisms.

Once formed, galaktóz1foszfátá is then converted to glucose-1-phosphate by the enzyme galactose-1-phosphate uridyltransferase (GALT). This enzyme requires UDP-glucose as a co-substrate, and the reaction involves the transfer of a uridyl group. The resulting glucose-1-phosphate can then enter the glycolytic pathway or be converted to glucose-6-phosphate for further metabolism.

Deficiencies in the GALT enzyme lead to classic galactosemia, a serious inherited metabolic disorder. In this condition, galaktóz1foszfátá accumulates in tissues, leading to toxic effects. Symptoms of untreated galactosemia include liver damage, cataracts, kidney problems, and intellectual disability. Newborn screening programs often test for galactosemia to enable early diagnosis and management, which typically involves a galactose-free diet. The understanding of galaktóz1foszfátá and its role in galactose metabolism is therefore vital for both basic biological research and clinical practice.