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AUTHOR
•Nimra khan
•The Women University,
Multan
Pakistan
TOPIC
“Glycolipids
”
GLYCOLIPIDS
• Glycolipids are glycoconjugates of lipids
• The term glycolipids designated any
compound containing one or more
monosaccharides bound by glycosidic
linkage.
• It is a structural lipid
OCCURANCE
•They are usually found on extracellular face
or eukaryotic cellular membrane
•glycolipids tend to aggregate to clusters in
aqueous environments, i. e., to lipid bilayers
or membranes.
THE MAJOR GLYCOLIPIDS FOUND IN ANIMAL
TISSUES ARE GLYCOSPHINGOLIPIDS
• They contain ceramide and one or more
sugars
• Ceramide + Glucose Glucocerebroside
• Ceramide + Galactose Galactoceribroside
SYNTHESIS OF GLYCOLIPIDS
• Synthesis of glycolipids occurs with the help of enzymes that
sequentially add sugars to the lipid.
• Glycolipids are synthesized in the Golgi-apparatus
• majority are transported to membranes to maintain the bilayer
• glycolipids can be found in the cystol approximately 5% of the
total glycolipids in the brain are found in the soluble fraction
TYPES OF
GLYCOLIPIDS
1)Cerebrosides-
• Cerebrosides (from cerebro=brain) are glycolipids that
are found primarily in the brain and peripheral (other
areas of the body) nervous tissue.
• Function-
Provide protective coating to each nerve and act
as insulator.
2)Gangliosides
These glycolipids ( glycosphingolipids )are
neutral. They are acidic in pH and are more complex of
glycolipids.
3) Sulfo glyco sphingolipids-
These cerebrosides are also called sulfatides, they are
simply cerebrosides with a sulphate residue on the sugar
portion of glycolipid. Occurrence This particular lipid is
found primarily in the medullated nerve fibers.
Cerebrosides glyco sphingolipids
Gangliosides
DEGRADATION OF
GLYCOLIPID
Lipids are required to breakdown, enzymes in
lysosomes of the cell help to remove the sugar
subunits.
 Metabolism of Glycolipid in Human The glycolipids are type
of lipids that is utilized by the human as well as other forms of
life.
 Glycolipids are derivatives of ceramides.
METABOLISM OF
GLYCOLIPID IN HUMAN
• The glycolipids are type of lipids that is
utilised by the human as well as other forms of
life.
• Glycolipids are derivatives of ceramides
• Glycolipids are simply fats that are bounded to
sugars.
FUNCTIONS
• Glycolipids are an essential part of cell membranes
• Glycolipids also help determine the blood group of an
individual.
• Glycolipids act as receptors at the surface of the red blood
cell.
• Some viruses, bacteria (e.g. Cholera) use glycolipids on
their cell surface as well.This helps the immune system
destroy and clear the pathogen from the body.
 It provide energy
•The antigens which determine blood types
belong glycolipids.
DISEASES
Lipid storage diseases
• They are called as sphingolipidoses
Gaucher's diseases
• Most common lysosomal storage diseases
• Enzyme deficiency --beta glucosidase
•Lipid accumulating-Glucosylceramide
•Clinical symptoms
3 types-adult, infantile, juvenile
Hepatosplenomegaly, erosion of long bones, moderate anemia,
mental retardation in infants.
Niemann-pick disease
enzyme deficiency – sphingomyelinase
Lipid accumulating-sphingomyelin
Clinical symptoms
Severe CNS damage, mental retardation hepatosplenomegaly,
cherry rod spot in macula
Neurodegenerative course(type A)
death occurs by 2 years of age
Krabbe's diseases Globoid cell dystrophy
•Enzyme deficiency- beta galactosidase
•Lipid accumulating-galactosyl ceramide
•Clinical symptoms
Serve mental retardation , total absence of
myeline in CNS, Globoid bodies in white matter
LABORATORY DIAGNOSIS
• A specific sphingolipidosis can be diagnosed by
measuring enzyme activity in cultured fibroblasts or
peripheral leukocytes or by analysis of DNA
• Histologic examination of the affected tissue is also
useful
All these diseases can be diagnosed
prenatally by amniocentesis and culture
of amniotic fluid cells
TREATMENT
• Replacement of deficient enzyme has bee tried in Gaucher's
diseases, with limited success
• Gaucher's diseases and Fabry diseases are treated by
recombinant human enzyme replacement therapy ,but the
monetary cost is extremely high
• Gaucher disease has also been treated by bone
marrow transplantation
• Other promoting approaches are substrate
deprivation therapy to inhibit the synthesis of
sphingolipid and chemical chaperone therapy
• Gene therapy for lysosomal disorder is also currently
under investigation
ANY
QUESTION
Glycolipids

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Glycolipids

  • 2. AUTHOR •Nimra khan •The Women University, Multan Pakistan
  • 4. GLYCOLIPIDS • Glycolipids are glycoconjugates of lipids • The term glycolipids designated any compound containing one or more monosaccharides bound by glycosidic linkage. • It is a structural lipid
  • 5. OCCURANCE •They are usually found on extracellular face or eukaryotic cellular membrane •glycolipids tend to aggregate to clusters in aqueous environments, i. e., to lipid bilayers or membranes.
  • 6. THE MAJOR GLYCOLIPIDS FOUND IN ANIMAL TISSUES ARE GLYCOSPHINGOLIPIDS • They contain ceramide and one or more sugars • Ceramide + Glucose Glucocerebroside • Ceramide + Galactose Galactoceribroside
  • 7.
  • 8. SYNTHESIS OF GLYCOLIPIDS • Synthesis of glycolipids occurs with the help of enzymes that sequentially add sugars to the lipid. • Glycolipids are synthesized in the Golgi-apparatus • majority are transported to membranes to maintain the bilayer • glycolipids can be found in the cystol approximately 5% of the total glycolipids in the brain are found in the soluble fraction
  • 9. TYPES OF GLYCOLIPIDS 1)Cerebrosides- • Cerebrosides (from cerebro=brain) are glycolipids that are found primarily in the brain and peripheral (other areas of the body) nervous tissue. • Function- Provide protective coating to each nerve and act as insulator.
  • 10. 2)Gangliosides These glycolipids ( glycosphingolipids )are neutral. They are acidic in pH and are more complex of glycolipids. 3) Sulfo glyco sphingolipids- These cerebrosides are also called sulfatides, they are simply cerebrosides with a sulphate residue on the sugar portion of glycolipid. Occurrence This particular lipid is found primarily in the medullated nerve fibers.
  • 13. DEGRADATION OF GLYCOLIPID Lipids are required to breakdown, enzymes in lysosomes of the cell help to remove the sugar subunits.  Metabolism of Glycolipid in Human The glycolipids are type of lipids that is utilized by the human as well as other forms of life.  Glycolipids are derivatives of ceramides.
  • 14. METABOLISM OF GLYCOLIPID IN HUMAN • The glycolipids are type of lipids that is utilised by the human as well as other forms of life. • Glycolipids are derivatives of ceramides • Glycolipids are simply fats that are bounded to sugars.
  • 15. FUNCTIONS • Glycolipids are an essential part of cell membranes • Glycolipids also help determine the blood group of an individual. • Glycolipids act as receptors at the surface of the red blood cell. • Some viruses, bacteria (e.g. Cholera) use glycolipids on their cell surface as well.This helps the immune system destroy and clear the pathogen from the body.
  • 16.  It provide energy •The antigens which determine blood types belong glycolipids.
  • 17. DISEASES Lipid storage diseases • They are called as sphingolipidoses Gaucher's diseases • Most common lysosomal storage diseases • Enzyme deficiency --beta glucosidase
  • 18. •Lipid accumulating-Glucosylceramide •Clinical symptoms 3 types-adult, infantile, juvenile Hepatosplenomegaly, erosion of long bones, moderate anemia, mental retardation in infants.
  • 19. Niemann-pick disease enzyme deficiency – sphingomyelinase Lipid accumulating-sphingomyelin Clinical symptoms Severe CNS damage, mental retardation hepatosplenomegaly, cherry rod spot in macula Neurodegenerative course(type A) death occurs by 2 years of age
  • 20. Krabbe's diseases Globoid cell dystrophy •Enzyme deficiency- beta galactosidase •Lipid accumulating-galactosyl ceramide •Clinical symptoms Serve mental retardation , total absence of myeline in CNS, Globoid bodies in white matter
  • 21. LABORATORY DIAGNOSIS • A specific sphingolipidosis can be diagnosed by measuring enzyme activity in cultured fibroblasts or peripheral leukocytes or by analysis of DNA • Histologic examination of the affected tissue is also useful
  • 22. All these diseases can be diagnosed prenatally by amniocentesis and culture of amniotic fluid cells
  • 23. TREATMENT • Replacement of deficient enzyme has bee tried in Gaucher's diseases, with limited success • Gaucher's diseases and Fabry diseases are treated by recombinant human enzyme replacement therapy ,but the monetary cost is extremely high
  • 24. • Gaucher disease has also been treated by bone marrow transplantation • Other promoting approaches are substrate deprivation therapy to inhibit the synthesis of sphingolipid and chemical chaperone therapy • Gene therapy for lysosomal disorder is also currently under investigation