Glycolipids are glycoconjugates containing lipids and sugars. They are typically found in cellular membranes and tend to cluster together. Major glycolipids in animal tissues include glycosphingolipids containing ceramide and one or more sugars. Glycolipids are synthesized in the Golgi apparatus and transported to membranes. Major types are cerebrosides, gangliosides, and sulfoglycosphingolipids. Glycolipids play roles in energy storage, determining blood type, and acting as receptors for pathogens. Diseases associated with glycolipids include lysosomal storage disorders due to enzyme deficiencies leading to accumulation of specific glycolipids. Diagnosis and treatment options include measuring enzyme activity, histological examination,
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
â˘They are usually found on extracellular face
or eukaryotic cellular membrane
â˘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 ď Glucocerebroside
⢠Ceramide + Galactose ď 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
ď§Lipids are required to breakdown, enzymes in
lysosomes of the cell help to remove the sugar
subunits.
ď§ Metabolism of Glycolipid in Human The glycolipids are type
of lipids that is utilized by the human as well as other forms of
life.
ď§ 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. ďą It provide energy
â˘The antigens which determine blood types
belong glycolipids.
17. DISEASES
ďąLipid storage diseases
⢠They are called as sphingolipidoses
ďąGaucher's diseases
⢠Most common lysosomal storage diseases
⢠Enzyme deficiency --beta glucosidase
19. ďąNiemann-pick disease
ďśenzyme deficiency â sphingomyelinase
ďśLipid accumulating-sphingomyelin
ďśClinical symptoms
ďźSevere CNS damage, mental retardation hepatosplenomegaly,
cherry rod spot in macula
ďźNeurodegenerative course(type A)
ďźdeath occurs by 2 years of age
20. ďąKrabbe's diseases Globoid cell dystrophy
â˘Enzyme deficiency- beta galactosidase
â˘Lipid accumulating-galactosyl ceramide
â˘Clinical symptoms
ďźServe 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. ďźAll 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