MUCOPOLYSACCHARIDES

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