Class 9 galactose metabolism
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Dr. Dhiraj J. Trivedi Biochemistry lectures for UG medical students, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, India.
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Class 9 galactose metabolism
- 2. Metabolism of Galactose • Major dietary source of galactose is Lactose • Lactose is digested by β-galactosidase (lactase) enzyme in intestine • Galactose uptake by cell is not influenced by Insulin • Galactose is required for glycoprotein, glycolipid and GAG synthesis • Lactose synthesis takes place in Mammary gland
- 3. Source of Galactose Lactose Galactose Lactase Lactase deficiency Intestinal mucosal cell Lactose intolerance Diarrhea, Vomiting, Nausea of milk Glucose
- 4. Phosphorylation of Galactose Galactose Galactose 1 Phosphate Galactokinase ATP ADP Mg+2 Galactokinase deficiency Rare Autosomal recessive disorder Increased Galactose in Blood Excretion of Galactose in Urine Galactitol NADP+ NADPH + H+ Aldose reductase
- 5. Reduction of Galactose Galactose When Galactose levels increases Galactose is reduced Increased level of Galactitol causes CATARACT Galactitol NADP+ NADPH + H+ Aldose reductase Aldose reductase is present in Liver, Kidney, Retina Lens, Nervous tissue, Seminal ves Ovaries
- 6. Synthesis of UDP-Galactose Galactose 1 Phosphate Galactose-1-P Uridyltransferase UTP PPi UDP- Galactose Galactose Classic Galactosemia Deficiency of Galactose 1-P Uridyl transferase Causes Galactosemia, Galactosuria, Vomiting, Diarrhea, Jaundice
- 7. Synthesis of UDP-Galactose Galactose 1 Phosphate Galactose-1-P Uridyltransferase UTP PPi UDP- GalactoseLactose Glycoprotein Glycolipid GAG UDP Glucose GlucoseUDP Lactose synthase UDP Hexose 4 epimerase Prolactin +
- 8. Galactosemia • Enzyme defect: • Galactose 1 P Uridyltransferase or Galactokinase •Inheritance : •Autosomal recessive •Age : Child
- 9. Galactosemia • Biochemical finding: Accumulation of Galactose in NT & Lens Galactosemia , Galactosuria, Hypoglycemia, • Clinical finding: • Failure to thrive on milk Liver disease, Mental retardation and CATARACT
- 10. Galactosemia • Diagnosis: • Reducing sugar in urine but GOD negative, Chromatography, Increased level of blood galactose • Treatment: • Galactose free diet