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Galactose metabolism, which converts galactose into glucose, is carried out by the three principal enzymes in a mechanism known as the Leloir pathway. The enzymes are listed in the order of the metabolic pathway: galactokinase (GALK), galactose-1-phosphate uridyltransferase (GALT), and UDP-galactose-4’-epimerase (GALE). [citation needed]
After separation from glucose, galactose travels to the liver for conversion to glucose. [12] Galactokinase uses one molecule of ATP to phosphorylate galactose. [2] The phosphorylated galactose is then converted to glucose-1-phosphate, and then eventually glucose-6-phosphate, which can be broken down in glycolysis. [2]
The Leloir pathway is a metabolic pathway for the catabolism of D-galactose.It is named after Luis Federico Leloir, who first described it. [1] [2] [3] [4]Intermediates and enzymes in the Leloir pathway of galactose metabolism [5]
Galactosemia, the inability to metabolize galactose in liver cells, is the most common monogenic disorder of carbohydrate metabolism, affecting 1 in every 55,000 newborns. [2] When galactose in the body is not broken down, it accumulates in tissues. The most common signs are failure to thrive, hepatic insufficiency, cataracts and developmental ...
Galactokinase is an enzyme (phosphotransferase) that facilitates the phosphorylation of α-D-galactose to galactose 1-phosphate at the expense of one molecule of ATP. [1] Galactokinase catalyzes the second step of the Leloir pathway, a metabolic pathway found in most organisms for the catabolism of α-D-galactose to glucose 1-phosphate. [2]
Galactosemia follows an autosomal recessive mode of inheritance that confers a deficiency in an enzyme responsible for adequate galactose degradation. Friedrich Goppert (1870–1927), a German physician, first described the disease in 1917, [1] with its cause as a defect in galactose metabolism being identified by a group led by Herman Kalckar ...
This is an accepted version of this page This is the latest accepted revision, reviewed on 11 December 2024. List of organ systems in the human body Part of a series of lists about Human anatomy General Features Regions Variations Movements Systems Structures Arteries Bones Eponymous Foramina Glands endocrine exocrine Lymphatic vessels Nerves Organs Systems Veins Muscles Abductors Adductors ...
319625 Ensembl ENSG00000143891 ENSMUSG00000035473 UniProt Q96C23 Q8K157 RefSeq (mRNA) NM_138801 NM_176963 RefSeq (protein) NP_620156 NP_795937 Location (UCSC) Chr 2: 38.67 – 38.74 Mb Chr 17: 80.43 – 80.49 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Galactose mutarotase (aldose 1-epimerase) (gene name GALM) is a human enzyme that reversibly converts α- aldose to the β ...