MUCOPOLYSACCHARIDES
I.SAMIYA BEGUM 28208203
MUCOPOLYSACCHARIDES - STRUCTURE
Heteropolysaccharides Linear polymers composed of repeating disaccharide units: One of the monosaccharide: N acetylglucosamine N acetylgalactosamine Other monosaccharide:
Uronic acid D glucuronic acid. L iduronic acid.
EXAMPLES OF MUCOPOLYSACCARIDES
1. Hyaluronic acid 2. Chondroitin sulfate 3. Heparin 4. Keratan sulfate 5. Dermatan sulfate
HYALURONIC ACID
o anionic, nonsulfated glycosaminoglycan. o Contain alternating residues of D
glucuronic acid
and N - acetylglucosamine o Linkage: N acetyl glucosamine beta 1, 4 - Glucuronic acid beta 1,3 N Acetyl glycosamine
HYALURONIC ACID
y FOUND IN: y Ground substances of synovial fluid of joints and vitreous humour of eyes. y Ground sustances in connective tissues y Forms gel around the ovum
HYALURONIC ACID
FUNCTION:
y a ubiquitous carbohydrate polymer which is a part
of the extracellular matrix. y a major component of the synovial fluid, which increases the viscosity of the fluid shock absorber. y major component of skin, which is involved in tissue repair.
DEGRADATION:
y HYALURONIDASES which degrades the
glucosidic linkage of Hyaluronic acid.
CHONDROITIN SULFATE
o sulfated glycosaminoglycan o Alterating units of
glucuronic acid beta 1,3 N acetyl galactosamine sulphate glucuronic acid.
CHONDROITIN SULFATE
FOUND IN:
It constitite the major components of various tissues like Bone Cartilage Tendons Heart Valves Skin cornea
FUNCTIONS
y major component of extracellular matrix, which
maintains the structural integrity of the tissue y chondroitin sulfate is a major component of cartilage loss of which cause of osteoarthritis. y In brain, it stabilize normal synapses.
HEPARIN
y a highly-sulfated glycosaminoglycan, is widely used as
an injectable anticoagulant. y N sulfoglucosamine 6-sulfate y alpha 1, 4 L iduronate 2 sulfate.
FUNCTIONS
y Naturally-occurring anticoagulant produced by
basophils and mast cells. y preventing the formation of clots and extension of existing clots within the blood y CONDITIONS used for anticoagulants:
y Acute coronary syndrome y heart surgery y Hemofiltration
FOUND IN ORGANS
yBlood yLung yLiver yKidney yspleen
DERMATAN SULFATE
y Alternating units of y L - iduronic acid beta 1, 3 N acetyl galactosamine 4 sulfate
DERMATAN SULFATE
FUNCTIONS: y Dermatan sulfate may have roles in coagulation,
cardiovascular disease, carcinogenesis, infection, wound repair, and fibrosis FOUND IN: y Mostly in the skin.
KERATAN SULFATE
y Only GAG that does not contains uronic acid y Repeating units of D - galactose and N
acetyl
glycosamine 6 sulfate in beta linkage
CLINICAL SIGNIFICANCE OF MUCOPOLYSACCHARIDES
y INBORN ERRORS:
Defect in enzymes which degrades the mucopolysaccharides leads to severe genetic disorder called Mucopolysaccharidoses
MUCOPOLYSACCHARIDOSES
y Lysosomal storage disease. y Either absence of 11 enzymes or defect in their
functioning. y 7 types of mucopolysaccharidoses
TYPES OF MUCOPOLYSACCHARIDOSES
TYPES Hurler syndrome Hunter syndrome Sanfilippo syndrome Morquio syndrome Maroteaux-Lamy syndrome Sly syndrome
ENZYME DEFECT
-L-iduronidase Iduronate sulfatase Heparan sulfamidase Beta-galactosidase N-acetylgalactosamine-4sulfatase -glucuronidase
ACCUMULATED PRODUCT
Heparan sulfate Dermatan sulfate Heparan sulfate Dermatan sulfate Heparan sulfate Keratan sulfate Dermatan sulfate Heparan sulfate Dermatan sulfate Chondroitin 4,6-sulfate
