D-Galactose

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D-Galactose
2D structure for D-Galactose
Chemical Name	alpha-D-galactopyranose
Chemical Formula	C₆H₁₂O₆
CAS Number	59-23-4
Chemical Information	HMDB00143
Biochemical Taxonomy	Not Available
Functional Taxonomy	Not Available
Nutritional Taxonomy	Carbohydrates
Metabolic Pathways	Galactose Metabolism Glycerolipid Metabolism Glycosphingolipid Metabolism
Biofluid Location	Blood Cellular Cytoplasm Cerebrospinal Fluid (CSF) Urine
Tissue Location	Liver Brain
Normal Biofluid Concentrations	Blood: 0.52 +/- 0.08 (0.12 -.1.25) uM Blood: 1.0 (0.0-5.0) uM Blood: 210.0 +/- 92.0 uM Blood: 39.0 +/- 12.0 uM Blood: 88.3 +/- 34.7 uM Cellular Cytoplasm: 4.6 (0.0-9.2) uM Cerebrospinal Fluid (CSF): 166.0 +/- 99.0 uM Urine: 262.58 +/- 881.72 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Patients with galactose-1-phosphate uridyltransferase deficiency galactosemia
Other (Monogenic Disorders)	Congenital disorder of glycosylation, type IA OMIM: 212065 Fanconi-bickel syndrome OMIM: 227810 Galactose epimerase deficiency OMIM: 230350 Galactosemia OMIM: 230400
Abnormal Biofluid Concentrations	Blood (Patients with galactose-1-phosphate uridyltransferase deficiency galactosemia): 2.72 +/- 0.70 (0.58 to 3.98) uM Cellular Cytoplasm (Galactosemia): 1496.5 (921.0 - 2070) uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
D-Galactose is an aldohexose that occurs naturally in the D-form in lactose, cerebrosides, gangliosides, and mucoproteins. D-Galactose is an energy-providing nutrient and also a necessary basic substrate for the biosynthesis of many macromolecules in the body. Metabolic pathways for D-Galactose are important not only for the provision of these pathways but also for the prevention of D-Galactose and D-Galactose metabolite accumulation. The main source of D-Galactose is lactose in the milk of mammals, but it can also be found in some fruits and vegetables. Utilization of D-Galactose in all living cells is initiated by the phosphorylation of the hexose by the enzyme galactokinase (E.C. 2.7.1.6) (GALK) to form D-Galactose-1-phosphate. In the presence of D-Galactose-1-phosphate uridyltransferase (E.C. 2.7.7.12) (GALT) D-Galactose-1-phosphate is exchanged with glucose-1-phosphate in UDP-glucose to form UDP-galactose. Glucose-1-phosphate will then enter the glycolytic pathway for energy production. Deficiency of the enzyme GALT in galactosemic patients leads to the accumulation of D-Galactose-1-phosphate. Classic galactosemia-a term that denotes the presence of D-Galactose in the blood is the rare inborn error of D-Galactose metabolism, diagnosed by the deficiency of the second enzyme of the D-Galactose assimilation pathway, GALT, which, in turn, is caused by mutations at the GALT gene. (PMID: 15256214, 11020650, 10408771)