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Metabolism
Of
Galactose
By
Dr. Santhosh Kumar N
Associate Professor of Biochemistry
Biomedical importance of
Galactose
Used as a liver function test (Galactose
tolerance test) the ability of the liver to convert
galactose to glucose
Synthesis of Glycolipids
(Cerebrosides & Gangliosides)
Synthesis of GAGs
(Keratan sulphate & Chondroitin
sulphate)
Synthesis of lactose
(lactating mammary gland)
Pentose
Pentose
Lactose
Lactase
Galactose
Galactose-1-phosphate
Gal-1-P-uridyl
transferase
Galactokinase ADP
Glucose-6-P
Glucose-1-Phosphate
GLUCOSE
ATP
Phosphoglucose
mutase
Glucose-6-phosphatase.
Pi
Energy
Synthesis of lactose
(lactating mammary gland)
Epimerase
UDP -Glucose –––––––––– UDP galactose
Lactose synthase
UDP -Galactose + glucose --------------------- Lactose
Disorders
Rare congenital disorder in infants due to accumulation
of galactose & galactose- 1-P in blood
Galactosaemia
Hypoglycaemia
Liver
Enlargement of liver
(due to malfunction of
liver)
Less uptake of
bilirubin &
reduced
conjugation
Jaundice
↑sed
Unconjugated
bilirubin
Galactitol (Dulcitol)
Aldose Reductase
Cataract: is a clouding of the lens
inside the eye which leads to a decrease
in vision
↓
Accumulates in lens
(due to its osmotic effect)
↓
congenital cataract
Deposited in renal tubules
Excreted galactose in urine
(glucosuria )
↓
Tubular damage
↓
Generalized amino aciduria
Diagnosis
• Congenital cataract, present of galactose in blood &
urine
• Collection of fetal cells by amniocentesis is useful in
prenatal diagnosis
Treatment
Removal of galactose from the diet
Amniocentesis :
• Removing of Amniotic fluid from the uterus for
testing or treatment.
• Amniotic fluid that surrounds and protects a baby
during pregnancy (contains fetal cells & various
proteins).
• Used primarily in prenatal diagnosis of
chromosomal abnormalities and fetal infections.
FRUCTOSE METABOLISM
Biomedical importance:
• Fructose rich in Seminal plasma & secreted by seminal vesicles
• Easily metabolized (by liver )
• Energy for mobility of spermatozoa derived from fructose
Fructose-1-phosphate
Fructokinase
Aldolase-B
Dihydroxyacetone
phosphate (DHAP)
Fructose
Glyceraldehyde
ADP
ATP
ESSENTIAL
FRUCTOSURIA ↑↑urine
No abnormality other than excretion of fructose in urine
Urine gives +ve benedicts test & Seliwanoff's test
Hereditary
fructose
intolerance
Autosomal recessive inborn error
Deficiency of Aldolase-B, leads to intracellular
trapping of fructose -1-P & Accumulation leads to
Hepatomegaly ,
Jaundice,
Renal dysfunctions, &
Hyperuricemia
Hereditary
fructose
intolerance
Depletes the ATP in cell
↓
Low ATP levels enhance oxidative
phosphorylation
↓
lowering of perfusion index levels in cell
(Remove the inhibitory effect on
adenosine deaminase )
↓
Increased rate of conversion of AMP to
IMP
↓
Uric acid
↓
Hyperuricemia
Hereditary
fructose
intolerance
Inhibits glycogen phosphorylase
↓
Inhibits glycogenolysis
(deposition of glycogen in liver)
↓
Severe Hypoglycemia
Vomiting and loss of appetite after
consumption of fructose containing food
Fru rapidly enters the tissues
↓
Enhanced fatty acid synthesis
↓
Raised serum TGs & ↑↑sed LDL-C levels in
blood
↓
Atherogenic & harmful conditions
Treatment : Removal of fructose & sucrose from the diet
Hereditary
fructose
intolerance
Diagnostic importance
• In azoospermia is seen due to a block in the duct.
• In such persons fructose is estimated in semen.
• If fructose is present the block is above the seminal vesicular duct;
• If absent block is after the seminal vesicles.
TIME IS FREE
BUT IT’S PRICELESS
You can’t own it
BUT YOU CAN USE IT
You cant keep it
BUT YOU CAN SPEND IT
Once you’ve lost it
YOU CAN NEVER GET IT
BACK

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