Autosomal Recessive

Accumulation of Heparan Sulfate (Mental Retardation)

Accumulation of Dermatan Sulfate (Skeletal Abnormalities)

Complete deficiency in a-L-IDURONIDASE

Death by ~age 10

X-Linked Recessive

Like, Hurler's -- Accumulation of Dermatan sulfate and Heparan sulfate

Deficiency in IDURONATE SULFATASE

Autosomal Recessive

Accumulation of Heparan Sulfate

Minor physical effects, but severe mental retardation

Types A, B, C, D

Deficiency in HEPARAN-N-SULFATASE

(same as HEPARAN SULFAMIDASE)

Unsulfated

NOT covalently linked to a protein core

Repeat unit is N-acetylglucosamine and glucuronic acid

Found in synovial fluid (joints) and vetrous humor (eye)

Serves as lubricant and shock absorber

Most Abundant GAG in body

Repeat unit is N-acetylgalactosamine and glucuronic acid

Can be sulfated at -4 &/or -6 position of N-acetylgalactosamine

Found in cartilage, tendons, ligaments, and aorta

Form proteoglycan aggregates, often aggregating noncovalently with hyaluronic acid

In cartilage, they bind collagen and hold fibers in a tight, strong network.

Repeat unit is N-acetylgalactosamine and Iduronic acid

Found in SKIN, Blood Vessels, and Heart Valves

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Repeat unit is N-acetylglucosamine and D-glucuronic acid or L-iduronic acid

Most highly charged of all GAGs

Heparin contains a-glycosidic bonds

(intracellular) Heparin is component of mast cells that line arteries (in liver, lungs, skin)

Acts as an anticoagulant and lipid-clearing agent

(extracellular) Heparan sulfate is found on the surface of many tissues including blood vessel walls and brain

Repeat unit is N-acetylglucosamine and galactose (no uronic acid)

Found in cornea and cartilage