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Fructose metabolism
โ€ข Dietary source is sucrose
โ€ข Fruits and honey are other sources of
fructose
โ€ข Absorption is not dependent on insulin
โ€ข It gets absorbed by GLUT 2 and GLUT
5 receptors
โ€ข Fructose is metabolized separately in
liver and muscle
โ€ข Too much of fructose leads to
hyperlipidemia
Metabolism of fructose
in muscle and extra hepatic tissue
Fructose
Fructose -6-phosphate
Glucose 6 Phosphate
ATP
ADP
Hexokinase
Pyruvate
Glycolysis
Glycogen
F1,6 BP
Phosphohexose isomerase
Metabolism of fructose
in Liver
Fructose
Fructose -1- phosphate
Glyceraldehyde
ATP
ADP
Fructokinase
TAG
Glycero phospholipid
Aldolase B
DHAP
Glycerol 3 P
Alcohol DH
Glycerol
NAD NAD H + H
Glycero kinase
ATP
ADP
Glycolysis
Gluconeogenesis
1
2
3
4
5
6
Inborn error involved in
Metabolism of fructose
Fructose
Fructose -1- phosphate
Glyceraldehyde
ATP
ADP
Fructokinase
Aldolase B
DHAP
Glycolysis / Gluconeogenesis
1
2
3
Deficiency of
fructokinase
Result in essential
Fructosuria
Deficiency of Aldolase B
Result in accumulation of F1P
Condition โ€“ Fructose
intolerance
Galactose metabolism
Metabolism of Galactose
โ€ข Major dietary source of galactose is Lactose
โ€ข Lactose is digested by ฮฒ-galactosidase
(lactase) enzyme in intestine
โ€ข Galactose uptake by cell is not influenced by
Insulin
โ€ข Galactose is required for glycoprotein,
glycolipid and GAG synthesis
โ€ข Lactose synthesis takes place in Mammary
gland
Source of Galactose
Lactose
Galactose
Lactase
Lactase deficiency
Intestinal mucosal cell
Lactose intolerance
Diarrhoea, Vomiting, Nausea of
milk
Glucose
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
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
Synthesis of UDP-Galactose
Galactose 1 Phosphate
Galactose-1-P
Uridyltransferase
UTP
PPi
UDP- Galactose
Classic Galactosemia
Deficiency of
Galactose 1-P Uridyl
transferase
Causes
Galactosemia,
Galactosuria,
Vomiting, Diarrhoea,
Jaundice
Galactose
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
+
Galactosemia
โ€ข Enzyme defect: Galactose 1 P Uridyltransferase or
Galactokinase
โ€ข Inheritance : Autosomal recessive
โ€ข Age : Child
โ€ข Biochemical finding:
Accumulation of Galactose in NT & Lens
Galactosemia , Galactosuria, Hypoglycemia,
โ€ข Clinical finding: Failure to thrive on milk
Liver disease, Mental retardation and CATARACT
โ€ข Diagnosis: Reducing sugar in urine but GOD negative,
Chromatography, Increased level of blood galactose
โ€ข Treatment: Galactose free diet

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Fructose & galactose metabolism

  • 1. Fructose metabolism โ€ข Dietary source is sucrose โ€ข Fruits and honey are other sources of fructose โ€ข Absorption is not dependent on insulin โ€ข It gets absorbed by GLUT 2 and GLUT 5 receptors โ€ข Fructose is metabolized separately in liver and muscle โ€ข Too much of fructose leads to hyperlipidemia
  • 2. Metabolism of fructose in muscle and extra hepatic tissue Fructose Fructose -6-phosphate Glucose 6 Phosphate ATP ADP Hexokinase Pyruvate Glycolysis Glycogen F1,6 BP Phosphohexose isomerase
  • 3. Metabolism of fructose in Liver Fructose Fructose -1- phosphate Glyceraldehyde ATP ADP Fructokinase TAG Glycero phospholipid Aldolase B DHAP Glycerol 3 P Alcohol DH Glycerol NAD NAD H + H Glycero kinase ATP ADP Glycolysis Gluconeogenesis 1 2 3 4 5 6
  • 4. Inborn error involved in Metabolism of fructose Fructose Fructose -1- phosphate Glyceraldehyde ATP ADP Fructokinase Aldolase B DHAP Glycolysis / Gluconeogenesis 1 2 3 Deficiency of fructokinase Result in essential Fructosuria Deficiency of Aldolase B Result in accumulation of F1P Condition โ€“ Fructose intolerance
  • 6. Metabolism of Galactose โ€ข Major dietary source of galactose is Lactose โ€ข Lactose is digested by ฮฒ-galactosidase (lactase) enzyme in intestine โ€ข Galactose uptake by cell is not influenced by Insulin โ€ข Galactose is required for glycoprotein, glycolipid and GAG synthesis โ€ข Lactose synthesis takes place in Mammary gland
  • 7. Source of Galactose Lactose Galactose Lactase Lactase deficiency Intestinal mucosal cell Lactose intolerance Diarrhoea, Vomiting, Nausea of milk Glucose
  • 8. 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
  • 9. 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
  • 10. Synthesis of UDP-Galactose Galactose 1 Phosphate Galactose-1-P Uridyltransferase UTP PPi UDP- Galactose Classic Galactosemia Deficiency of Galactose 1-P Uridyl transferase Causes Galactosemia, Galactosuria, Vomiting, Diarrhoea, Jaundice Galactose
  • 11. 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 +
  • 12. Galactosemia โ€ข Enzyme defect: Galactose 1 P Uridyltransferase or Galactokinase โ€ข Inheritance : Autosomal recessive โ€ข Age : Child โ€ข Biochemical finding: Accumulation of Galactose in NT & Lens Galactosemia , Galactosuria, Hypoglycemia, โ€ข Clinical finding: Failure to thrive on milk Liver disease, Mental retardation and CATARACT โ€ข Diagnosis: Reducing sugar in urine but GOD negative, Chromatography, Increased level of blood galactose โ€ข Treatment: Galactose free diet