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MUCOPOLYSACCHARIDESMUCOPOLYSACCHARIDES -- STRUCTURESTRUCTURE

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.

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EXAMPLES OF MUCOPOLYSACCARIDESEXAMPLES OF MUCOPOLYSACCARIDES

1. Hyaluronic acid

2. Chondroitin sulfate3. Heparin

4. Keratan sulfate5. Dermatan sulfate

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HYALURONIC ACIDHYALURONIC ACID

o anionic, nonsulfated glycosaminoglycan.

o Contain alternating residues ofD glucuronic acid

and N - acetylglucosamineo Linkage: N acetyl glucosamine

beta 1, 4 - Glucuronic acid

beta 1,3 N Acetyl glycosamine

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HYALURONIC ACIDy FOUND IN:

y Ground substances of synovial f luid of joints and

vitreous humour of eyes.y Ground sustances in connective tissues

y Forms gel around the ovum

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HYALURONIC ACID

FUNCTION:y a ubiquitous carbohydrate polymer which is a part

of the extracellular matrix.

y a major component of the synovial f luid, whichincreases the viscosity of the f luid shockabsorber.

y major component of skin, which is involved intissue repair.

DEGRADATION:

yHYALURONIDASES which degrades theglucosidic linkage of Hyaluronic acid.

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CHONDROITIN SULFATECHONDROITIN SULFATEo sulfated glycosaminoglycan

oAlterating units of

glucuronic acid beta 1,3 N acetylgalactosamine sulphate glucuronic acid.

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CHONDROITINCHONDROITIN SULFATESULFATEFOUND IN:

It constitite the major components of various tissues like

Bone Cartilage

Tendons

Heart

Valves Skin

cornea

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FUNCTIONSFUNCTIONSy major component of extracellular matrix, which

maintains the structural integrity of the tissue

y chondroitin sulfate is a major component of cartilageloss of which cause of osteoarthritis.

y In brain, it stabilize normal synapses.

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HEPARINHEPARINy a highly-sulfated glycosaminoglycan, is widely used as

an injectable anticoagulant.

yN sulfoglucosamine 6-sulfate

y alpha 1, 4 L iduronate 2 sulfate.

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DERMATAN SULFATEyAlternating units of

y L - iduronic acid beta 1, 3 N acetyl galactosamine

4 sulfate

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DERMATAN SULFATEFUNCTIONS:

y Dermatan sulfate may have roles in coagulation,

cardiovascular disease, carcinogenesis, infection,wound repair, and fibrosis

FOUND IN:

y Mostly in the skin.

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KERATAN SULFATE

y Only GAG that does not contains uronic acid

y Repeating units of D - galactose and N acetylglycosamine 6 sulfate in beta linkage

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MUCOPOLYSACCHARIDOSESMUCOPOLYSACCHARIDOSESy Lysosomal storage disease.

y Either absence of 11 enzymes or defect in their

functioning.y 7 types of mucopolysaccharidoses

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TYPES OF MUCOPOLYSACCHARIDOSESTYPES OF MUCOPOLYSACCHARIDOSES

TYPES ENZYME DEFECT ACCUMULATEDPRODUCT

Hurler syndrome -L-iduronidase Heparan sulfateDermatan sulfate

Hunter syndrome Iduronate sulfatase Heparan sulfate

Dermatan sulfate

Sanfilippo syndrome Heparan sulfamidase Heparan sulfate

Morquio syndrome Beta-galactosidase Keratan sulfate

Maroteaux-Lamysyndrome

N-acetylgalactosamine-4-sulfatase

Dermatan sulfate

Sly syndrome -glucuronidase Heparan sulfateDermatan sulfateChondroitin 4,6-sulfate

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